Document:

EX-4.3

 Exhibit 4.3 

UNLESS THIS SECURITY IS PRESENTED BY AN AUTHORIZED REPRESENTATIVE OF THE DEPOSITORY TRUST COMPANY TO THE COMPANY OR ITS AGENT FOR REGISTRATION OF TRANSFER,
EXCHANGE OR PAYMENT, AND ANY SECURITY ISSUED IS REGISTERED IN THE NAME OF CEDE & CO. OR IN SUCH OTHER NAME AS IS REQUESTED BY AN AUTHORIZED REPRESENTATIVE OF THE DEPOSITORY TRUST COMPANY (AND ANY PAYMENT HEREON IS MADE TO CEDE &
CO. OR TO SUCH OTHER ENTITY AS IS REQUESTED BY AN AUTHORIZED REPRESENTATIVE OF THE DEPOSITORY TRUST COMPANY), ANY TRANSFER, PLEDGE OR OTHER USE HEREOF FOR VALUE OR OTHERWISE BY OR TO ANY PERSON IS WRONGFUL SINCE THE REGISTERED OWNER HEREOF,
CEDE & CO., HAS AN INTEREST HEREIN. 
 TRANSFERS OF THIS SECURITY SHALL BE LIMITED TO TRANSFERS IN WHOLE, BUT NOT IN PART, TO NOMINEES OF
CEDE & CO. OR TO A SUCCESSOR THEREOF OR SUCH SUCCESSOR’S NOMINEE. 
 CSX CORPORATION 

$450,000,000 
 4.500% NOTES DUE
2054 
  

			
	No. R-1	  	CUSIP No. 126408 HA4

 This security (the “Security”) is one of a duly authorized issue of securities (herein called the
“Securities”) of CSX Corporation, a Virginia corporation (hereinafter called the “Company,” which term includes any successor corporation under the Indenture hereinafter referred to), issued and to be issued in one or more series
under an indenture, unlimited as to aggregate principal amount, dated as of August 1, 1990 between the Company and The Bank of New York Mellon Trust Company, N.A. (formerly known as The Bank of New York Trust Company, N.A.), successor to
JPMorgan Chase Bank, N.A. (formerly known as The Chase Manhattan Bank), as Trustee (herein called the “Trustee,” which term includes any successor trustee under the Indenture (as hereinafter defined)), as supplemented by a First
Supplemental Indenture dated as of June 15, 1991, a Second Supplemental Indenture dated as of May 6, 1997, a Third Supplemental Indenture dated as of April 22, 1998, a Fourth Supplemental Indenture dated as of October 30, 2001, a
Fifth Supplemental Indenture dated as of October 27, 2003, a Sixth Supplemental Indenture dated as of September 23, 2004, a Seventh Supplemental Indenture dated as of April 25, 2007 and an Eighth Supplemental Indenture dated as of
March 24, 2010, to which indenture and all indentures supplemental thereto (the indenture, as supplemented being herein called the “Indenture”) reference is hereby made for a statement of the respective rights thereunder of the
Company, the Trustee and the Holders of the Securities and of the terms upon which the Securities are, and are to be, authenticated and delivered. This Security is one of the series designated on the face hereof, which series has been issued in an
initial aggregate principal amount of $450,000,000 (FOUR HUNDRED FIFTY MILLION DOLLARS). All Securities of 

 
this series need not be issued at the same time and such series may be reopened at any time, without the consent of any Holder, for issuances of additional Securities of this series. Any such
additional Securities of this series will have the same interest rate, maturity and other terms as those initially issued. Further Securities of this series may also be authenticated and delivered as provided by Sections 304, 305, 306 or 906 of
the Indenture. This Security represents an aggregate initial principal amount of $450,000,000 (FOUR HUNDRED FIFTY MILLION DOLLARS) (adjusted from time to time in accordance with the terms and provisions hereof and as set forth on Exhibit A hereto,
the “Principal Amount”) of the Securities of such series, with the Interest Payment Dates, date of original issuance and date of Maturity specified herein and bearing interest on said Principal Amount at the interest rate specified herein.

 The Company, for value received, hereby promises to pay CEDE & CO., or its registered assigns, the principal sum of $450,000,000
(FOUR HUNDRED FIFTY MILLION DOLLARS) on August 1, 2054, and to pay interest (computed on the basis of a 360-day year of twelve 30-day months) thereon from
July 21, 2014 or from the most recent Interest Payment Date to which interest has been paid or duly provided for, or, if the date of this Security is an Interest Payment Date to which interest has been paid or duly provided for, then from the
date hereof, semiannually in arrears on February 1 and August 1 of each year, commencing February 1, 2015, and at Maturity at the rate of 4.500% per annum, until the principal hereof is paid or duly made available for payment.
The Company shall pay interest on overdue principal and premium, if any, and (to the extent lawful) interest on overdue installments of interest at the rate per annum borne by the Security. The interest so payable, and punctually paid or duly
provided for, on any Interest Payment Date will, as provided in such Indenture, be paid to the Person in whose name this Security (or one or more Predecessor Securities) is registered at the close of business on the Regular Record Date for such
interest, which shall be the January 15 or July 15 (whether or not a Business Day), as the case may be, next preceding such Interest Payment Date. Except as otherwise provided in the Indenture, any such interest not so punctually paid or
duly provided for shall forthwith cease to be payable to the Holder on such Regular Record Date and may be paid to the Person in whose name this Security (or one or more Predecessor Securities) is registered at the close of business on a Special
Record Date to be fixed by the Trustee for the payment of such Defaulted Interest, notice whereof shall be given to the Holder of this Security not less than 10 days prior to such Special Record Date, or may be paid at any time in any other
lawful manner not inconsistent with the requirements of any securities exchange or automated quotation system on which the Securities of this series may be listed or quoted, and upon such notice as may be required by such exchange or system, all as
more fully provided in such Indenture. Notwithstanding the foregoing, interest payable on this Security at Maturity will be payable to the person to whom principal is payable. 

This Security is exchangeable in whole or from time to time in part for definitive Registered Securities of this series only as provided in
this paragraph. If (x) the Depository with respect to the Securities of this series (the “Depository”) notifies the Company that it is unwilling, unable or ineligible to continue as Depository for this Security or if at any time
the Depository ceases to be a clearing agency registered under the Securities Exchange Act of 1934, as amended, and a successor Depository is not appointed by the Company within 90 days, (y) the Company in its sole discretion determines
that this Security shall be exchangeable for definitive Registered Securities and executes and delivers to the Trustee a Company Order providing that this Security shall be so exchangeable or (z) there shall have happened and be continuing an

 
Event of Default or any event which, after notice or lapse of time, or both, would become an Event of Default with respect to the Securities of the series of which this Security is a part, this
Security or any portion hereof shall, in the case of clause (x) above, be exchanged for definitive Registered Securities of this series, and in the case of clauses (y) and (z) above, be exchangeable for definitive Registered
Securities of this series, provided that the definitive Security so issued in exchange for this Security shall be in authorized denominations and be of like tenor and of an equal aggregate principal amount as the portion of the Security to be
exchanged, and provided further that, in the case of clauses (y) and (z) above, definitive Registered Securities of this series will be issued in exchange for this Security, or any portion hereof, only if such definitive Registered
Securities were requested by written notice to the Security Registrar by or on behalf of a Person who is a beneficial owner of an interest herein given through the Holder hereof. Any definitive Registered Security of this series issued in exchange
for this Security, or any portion hereof, shall be registered in the name or names of such Person or Persons as the Holder hereof shall instruct the Security Registrar. Except as provided above, owners of beneficial interests in this Security will
not be entitled to receive physical delivery of Securities in definitive form and will not be considered the Holders thereof for any purpose under the Indenture. 

Any exchange of this Security or portion hereof for one or more definitive Registered Securities of this series will be made at the New York
office of the Security Registrar or at the office of any transfer agent designated by the Company for that purpose. Upon exchange of any portion of this Security for one or more definitive Registered Securities of this series, the Trustee shall
endorse Exhibit A of this Security to reflect the reduction of its Principal Amount by an amount equal to the aggregate principal amount of the definitive Registered Securities of this series so issued in exchange, whereupon the Principal Amount
hereof shall be reduced for all purposes by the amount so exchanged and noted. Except as otherwise provided herein or in the Indenture, until exchanged in full for one or more definitive Registered Securities of this series, this Security shall in
all respects be subject to and entitled to the same benefits and conditions under the Indenture as a duly authenticated and delivered definitive Registered Security of this series. 

The principal and any interest in respect of any portion of this Security payable in respect of an Interest Payment Date or at the Stated
Maturity thereof, in each case occurring prior to the exchange of such portion for a definitive Registered Security or Securities of this series, will be paid, as provided herein, to the Holder hereof which will undertake in such circumstances to
credit any such principal and interest received by it in respect of this Security to the respective accounts of the Persons who are the beneficial owners of such interests on such Interest Payment Date or at Stated Maturity. If a definitive
Registered Security or Registered Securities of this series are issued in exchange for any portion of this Security after the close of business at the office or agency where such exchange occurs on (i) any Regular Record Date and before the
opening of business at such office or agency on the relevant Interest Payment Date or (ii) any Special Record Date and before the opening of business at such office or agency on the related proposed date for payment of Defaulted Interest, then
interest or Defaulted Interest, as the case may be, will not be payable on such Interest Payment Date or proposed date for payment, as the case may be, in respect of such Registered Security, but will be payable on such Interest Payment Date or
proposed date for payment, as the case may be, only to the Holder hereof, and the Holder hereof will undertake in such circumstances to credit such interest to the account or accounts of the Persons who were the beneficial owners of such portion of
this Security on such Regular Record Date or Special Record Date, as the case may be. 

 Payment of the principal of and any such interest on this Security will be made at the offices of
the Trustee as Paying Agent, in the Borough of Manhattan, The City of New York, or at such other office or agency of the Company as may be designated by it for such purpose in the Borough of Manhattan, The City of New York, in such coin or currency
of the United States of America as at the time of payment shall be legal tender for the payment of public and private debts by check mailed to the registered Holders thereof; provided, however, that at the option of the Holder, payment of interest
may be made by wire transfer of immediately available funds to an account of the Person entitled hereto as such account shall be provided to the Security Registrar and shall appear in the Security Register. 

The Securities shall be redeemable, in whole or in part, at the Company’s option at any time. If the Securities are redeemed prior to the
date that is six months prior to the Maturity date, the Redemption Price for the Securities to be redeemed shall equal the greater of the following amounts, plus, in each case, accrued interest thereon to the Redemption Date: 

 

	 	•	 	100% of the principal amount of such Securities; or 

  

	 	•	 	as determined by the Independent Investment Banker (as defined below), the sum of the present values of the remaining scheduled payments of principal and interest on the Securities to be redeemed (not including any
portion of any payments of interest accrued from the most recent Interest Payment Date to which interest has been paid to the Redemption Date) discounted to the Redemption Date on a semiannual basis at the Adjusted Treasury Rate (as defined below)
plus 20 basis points. 

 If the Securities are redeemed on or after the date that is six months prior to the Maturity date,
the redemption price for the Securities to be redeemed will equal 100% of the principal amount of such Securities, plus accrued interest to the Redemption Date. 
  

	 	•	 	The Redemption Price shall be calculated by the Independent Investment Banker assuming a 360-day year consisting of twelve 30-day months.

 “Adjusted Treasury Rate” means, with respect to any Redemption Date: 

 

	 	•	 	the yield, under the heading which represents the average for the immediately preceding week, appearing in the most recently published statistical release designated “H.15(519)” or any successor publication
which is published weekly by the Board of Governors of the Federal Reserve System and which establishes yields on actively traded U.S. Treasury securities adjusted to constant maturity under the caption “Treasury Constant Maturities,” for
the maturity corresponding to the Comparable Treasury Issue (if no maturity is within three months before or after the remaining term of the Securities to be redeemed, yields for the two published maturities most closely corresponding to the
Comparable Treasury Issue will be determined and the Adjusted Treasury Rate will be interpolated or extrapolated from such yields on a straight-line basis, rounding to the nearest month); or 

	 	•	 	if such release (or any successor release) is not published during the week preceding the calculation date or does not contain such yields, the rate per annum equal to the semiannual equivalent yield to maturity of the
Comparable Treasury Issue, assuming a price for the Comparable Treasury Issue (expressed as a percentage of its principal amount) equal to the Comparable Treasury Price for such Redemption Date. 

The Adjusted Treasury Rate shall be calculated on the third Business Day preceding the Redemption Date. The Company shall notify the Trustee,
in an Officers’ Certificate, of the Redemption Price no later than the second Business Day preceding the Redemption Date. The Officers’ Certificate shall set forth the Redemption Price both as an aggregate amount for all the Securities to
be redeemed and as an amount per $1,000 in principal amount of the Securities to be redeemed, subject to a minimum $2,000 denomination as set forth below. 

“Comparable Treasury Issue” means the U.S. Treasury security selected by the Independent Investment Banker as having a maturity
comparable to the remaining term of the Securities to be redeemed that would be utilized, at the time of selection and in accordance with customary financial practice, in pricing new issues of corporate debt securities of comparable maturity to the
remaining term of such Securities. 
 “Comparable Treasury Price” means, with respect to any Redemption Date, (A) the average
of five Reference Treasury Dealer Quotations for such Redemption Date, after excluding the highest and lowest such Reference Treasury Dealer Quotations, or (B) if the Independent Investment Banker obtains fewer than five such Reference Treasury
Dealer Quotations, the average of all such quotations. 
 “Independent Investment Banker” means Citigroup Global Markets Inc.,
Credit Suisse Securities (USA) LLC, Morgan Stanley & Co. LLC or UBS Securities LLC and their successors, or if they are unwilling or unable to serve in that capacity, an independent investment and banking institution of national standing
appointed by the Company. 
 “Reference Treasury Dealer” means each of: 

 

	 	•	 	Citigroup Global Markets Inc., Credit Suisse Securities (USA) LLC, Morgan Stanley & Co. LLC and UBS Securities LLC and their successors; provided that, if any ceases to be a primary U.S. Government securities
dealer in the United States (“Primary Treasury Dealer”), the Company will substitute another Primary Treasury Dealer; and 

  

	 	•	 	up to four other Primary Treasury Dealers selected by the Company. 

 “Reference Treasury
Dealer Quotation” means, with respect to each Reference Treasury Dealer and any Redemption Date, the average, as determined by the Independent Investment Banker, of the bid and asked prices for the Comparable Treasury Issue (expressed in each
case as 

 
a percentage of its principal amount) quoted in writing to the Independent Investment Banker by such Reference Treasury Dealer at 5:00 p.m. (New York City time) on the third business day
preceding such Redemption Date. 
 Notice of redemption shall be given as provided in Section 1104 of the Indenture; provided, that
such notice shall not be required to include the Redemption Price but shall instead include the manner of calculation of the Redemption Price. If the Company elects to partially redeem the Securities, the Trustee will select in a fair and
appropriate manner the Securities to be redeemed. 
 Unless the Company defaults in payment of the Redemption Price, on and after the
Redemption Date interest will cease to accrue on the Securities or portions thereof called for redemption. 
 If an Event of Default with
respect to Securities of this series shall occur and be continuing, the principal of the Securities of this series (including this Security and the interests represented hereby) may be declared due and payable in the manner and with the effect
provided in the Indenture. Upon payment (i) of the amount of principal so declared due and payable and (ii) of interest on any overdue principal and overdue interest (in each case to the extent that the payment of such interest shall be
legally enforceable), all of the Company’s obligations in respect of the payment of the principal of and any interest on the Securities of this series (including this Security and the interests represented hereby) shall terminate. 

If a Change of Control Repurchase Event occurs, unless the Company has exercised the Company’s right to redeem the Securities as
described above, the Company will be required to make an offer to each Holder of the Securities to repurchase all or any part (equal to $2,000 or an integral multiple of $1,000 in excess thereof) of that Holder’s Securities at a repurchase
price in cash equal to 101% of the aggregate principal amount of the Securities repurchased plus any accrued and unpaid interest on the Securities repurchased to, but not including, the date of repurchase. Within 30 days following any Change of
Control Repurchase Event or, at the Company’s option, prior to any Change of Control, but after the public announcement of the Change of Control, the Company will mail a notice to each Holder, with a copy to the Trustee, describing the
transaction or transactions that constitute or may constitute the Change of Control Repurchase Event and offering to repurchase the Securities on the payment date specified in the notice, which date will be no earlier than 30 days and no later
than 60 days from the date such notice is mailed. The notice shall, if mailed prior to the date of consummation of the Change of Control, state that the offer to purchase is conditioned on a Change of Control Repurchase Event occurring on or
prior to the payment date specified in the notice. The Company will comply with the requirements of Rule 14e-1 under the Exchange Act, and any other securities laws and regulations thereunder to the
extent those laws and regulations are applicable in connection with the repurchase of the Securities as a result of a Change of Control Repurchase Event. To the extent that the provisions of any securities laws or regulations conflict with the
Change of Control Repurchase Event provisions of the Securities, the Company will comply with the applicable securities laws and regulations and will not be deemed to have breached the Company’s obligations under the Change of Control
Repurchase Event provisions of the Securities by virtue of such conflict or compliance. 

 On the repurchase date following a Change of Control Repurchase Event, the Company will, to the
extent lawful: 
  

	 	(1)	accept for payment all the Securities or portions of the Securities properly tendered pursuant to the Company’s offer; 

  

	 	(2)	deposit with the paying agent an amount equal to the aggregate purchase price in respect of all the Securities or portions of the Securities properly tendered; and 

 

	 	(3)	deliver or cause to be delivered to the Trustee the Securities properly accepted, together with an officers’ certificate stating the aggregate principal amount of the Securities being purchased by the Company.

 The paying agent will promptly mail to each holder of the Securities properly tendered the purchase price for the
Securities, and the Trustee will promptly authenticate and mail (or cause to be transferred by book-entry) to each Holder a new note equal in principal amount to any unpurchased portion of any Securities
surrendered; provided that each new note will be in a principal amount of $2,000 or an integral multiple of $1,000 in excess thereof. 
 The
Company will not be required to make an offer to repurchase the Securities upon a Change of Control Repurchase Event if a third party makes such an offer in the manner, at the times and otherwise in compliance with the requirements for an offer made
by the Company and such third party purchases all the Securities properly tendered and not withdrawn under its offer. 
 For purposes of the
foregoing description of a repurchase at the option of holders, the following definitions are applicable: 
 “Below Investment Grade
Ratings Event” means that on any day within the 60-day period (which period shall be extended so long as the rating of the Securities is under publicly announced consideration for a possible downgrade by
any of the Rating Agencies) after the earlier of (1) the occurrence of a Change of Control; or (2) public notice of the occurrence of a Change of Control or the intention by the Company to effect a Change of Control, the Securities are
rated below Investment Grade by each of the Rating Agencies. Notwithstanding the foregoing, a Below Investment Grade Ratings Event otherwise arising by virtue of a particular reduction in rating shall not be deemed to have occurred in respect of a
particular Change of Control (and thus shall not be deemed a Below Investment Grade Ratings Event for purposes of the definition of Change of Control Repurchase Event hereunder) if the Rating Agencies making the reduction in rating to which this
definition would otherwise apply do not announce or publicly confirm or inform the Trustee in writing at its request that the reduction was the result, in whole or in part, of any event or circumstance comprised of or arising as a result of, or in
respect of, the applicable Change of Control (whether or not the applicable Change of Control shall have occurred at the time of the ratings event). 

“Change of Control” means the consummation of any transaction (including, without limitation, any merger or consolidation) the
result of which is that any “person” (as that term is used in Section 13(d)(3) of the Exchange Act), other than the Company or the Company’s subsidiaries, becomes the beneficial owner (as defined in
Rules 13d-3 and 13d-5 under the Exchange Act), directly or indirectly, of more than 50% of the combined voting power of the

 
Company’s Voting Stock or other Voting Stock into which the Company’s Voting Stock is reclassified, consolidated, exchanged or changed, measured by voting power rather than number of
shares. 
 “Change of Control Repurchase Event” means the occurrence of both a Change of Control and a Below Investment Grade
Ratings Event. 
 “Investment Grade” means a rating of Baa3 or better by Moody’s (or its equivalent under any successor
rating categories of Moody’s); a rating of BBB- or better by S&P (or its equivalent under any successor rating categories of S&P); or the equivalent Investment Grade credit rating from any additional Rating Agency or Rating Agencies
selected by the Company. 
 “Moody’s” means Moody’s Investors Service, Inc. 

“Rating Agency” means (1) each of Moody’s and S&P; and (2) if any of Moody’s or S&P ceases to rate the
Securities or fails to make a rating of the Securities publicly available for reasons outside of the Company’s control, a “nationally recognized statistical rating organization” as defined in Section 3(a)(62) of the Exchange Act,
selected by the Company (as certified by a resolution of the Chief Executive Officer or Chief Financial Officer) as a replacement agency for Moody’s or S&P, or both of them, as the case may be. 

“S&P” means Standard & Poor’s Ratings Services, a division of McGraw-Hill
Financial, Inc. 
 “Voting Stock” of any specified “person” (as that term is used in Section 13(d)(3) of the
Exchange Act) as of any date means the capital stock of such person that is at the time entitled to vote generally in the election of the board of directors of such person. 

The Trustee shall be entitled to deduct FATCA Withholding Tax that it reasonably determines it is required to deduct, and shall have no
obligation to gross-up any payment hereunder or to pay any additional amount as a result of such FATCA Withholding Tax. 
 For purposes of
the foregoing discussion of matters concerning the Trustee, the following definitions are applicable: 
 “FATCA Withholding Tax”
means any withholding or deduction pursuant to an agreement described in Section 1471(b) of the Code or otherwise imposed pursuant to Sections 1471 through 1474 of the Code (or any regulations or agreements thereunder or official
interpretations thereof) or any intergovernmental agreement between the United States and another jurisdiction facilitating the implementation thereof (or any law implementing such an intergovernmental agreement). 

“Code” means the U.S. Internal Revenue Code of 1986, as amended. 

The Indenture contains provisions for defeasance at any time of (a) the entire indebtedness of the Company on this Security and
(b) certain restrictive covenants and the related defaults and Events of Default, upon compliance with certain conditions set forth therein, which provisions shall apply to this Security. 

 The provisions of Article Fourteen of the Indenture apply to Securities of this series. 

The Indenture permits, with certain exceptions as therein provided, the amendment thereof and the modification of the rights and obligations
of the Company and the rights of the Holders of the Securities of each series to be affected under the Indenture at any time by the Company and the Trustee with the consent of the Holders of a majority in aggregate principal amount of the Securities
at the time Outstanding of each series affected thereby. The Indenture also contains provisions permitting the Holders of specified percentages in aggregate principal amount of the Securities of each series at the time Outstanding on behalf of the
Holders of all Securities of such series to waive compliance by the Company with certain provisions of the Indenture and certain past defaults under the Indenture and their consequences. Any such consent or waiver by the Holder of this Security
shall be conclusive and binding upon such Holder and upon all future Holders of this Security and the Persons who are beneficial owners of interests represented hereby, and of any Security issued in exchange herefor or in lieu hereof, whether or not
notation of such consent or waiver is made upon this Security. 
 As set forth in, and subject to, the provisions of the Indenture, no
Holder of any Security of this series will have any right to institute any proceeding with respect to the Indenture or for any remedy thereunder, unless such Holder shall have previously given to the Trustee written notice of a continuing Event of
Default with respect to the Securities of this series, the Holders of not less than 25% in aggregate principal amount of the Outstanding Securities of this series shall have made written request, and offered reasonable indemnity, to the Trustee to
institute such proceeding as trustee and the Trustee shall not have received from the Holders of a majority in aggregate principal amount of the Outstanding Securities of this series a direction inconsistent with such request and shall have failed
to institute such proceeding within 60 days; provided, however, that such limitations do not apply to a suit instituted by the Holder hereof for the enforcement of payment of the principal of (and premium, if any) or interest on this Security
on or after the respective due dates expressed herein. 
 No reference herein to the Indenture and no provision of this Security or of the
Indenture shall alter or impair the obligation of the Company, which is absolute and unconditional, to pay the principal of (and premium, if any) and interest on this Security at the time, place and rate, and in the coin or currency, herein
prescribed. 
 As provided in the Indenture and subject to certain limitations therein and herein set forth, the transfer of Registered
Securities of the series of which this Security is a part may be registered on the Security Register of the Company, upon surrender of such Securities for registration of transfer at the office of the Security Registrar, duly endorsed by, or
accompanied by a written instrument of transfer in form satisfactory to the Company and the Security Registrar duly executed by the Holder thereof or his attorney duly authorized in writing, and thereupon one or two more new Securities of this
series and of like tenor, of authorized denominations and for the same aggregate principal amount, will be issued to the designated transferee or transferees. 

No service charge shall be made for any such registration of transfer or exchange of Securities as provided above, but the Company may require
payment of a sum sufficient to cover any tax or other governmental charge payable in connection therewith. 

 Prior to due presentment of this Security for registration of transfer, the Company, the Trustee
and any agent of the Company or the Trustee may treat the Person in whose name this Security is registered as the owner hereof for all purposes, whether or not this Security be overdue, and neither the Company, the Trustee nor any such agent shall
be affected by notice to the contrary. 
 The Securities of this series of which this Security is a part are issuable only in registered
form without coupons, in denominations of $2,000 and integral multiples of $1,000. As provided in the Indenture and subject to certain limitations therein set forth, the Securities of this series are exchangeable for a like aggregate principal
amount of Securities of this series and of like tenor of a different authorized denomination, as requested by the Holder surrendering the same. 

The Securities of this series shall be dated the date of their authentication. 

All terms used in this Security which are defined in the Indenture shall have the meanings assigned to them in the Indenture. 

Unless the certificate of authentication hereon has been executed by or on behalf of the Trustee under the Indenture, or its successor
thereunder, by the manual signature of one of its authorized officers, this Security shall not be entitled to any benefit under the Indenture or be valid or obligatory for any purpose. 

 IN WITNESS WHEREOF, the Company has caused this instrument to be duly executed under its
corporate seal. 
  

							
	Dated: July     , 2014	 		 	CSX CORPORATION
				
		 		 	By:	 	  

		 		 		 	Name:
		 		 		 	Title:

 Attest: 

Assistant Corporate Secretary 
  

					
	District of Columbia	  	)	 	ss.:
		  	)	 	

 Before me, a Notary Public in and for said State and County/City, personally appeared
                                        ,
personally known to me or proved to me on the basis of satisfactory evidence to be the person whose name is subscribed to the within instrument and acknowledged to me that he executed the same in his authorized capacity, and that by his signature on
the instrument, the company on behalf of which he acted executed the instrument. 
 WITNESS my hand and official seal this day
     of July, 2014, in the State and County/City aforesaid. 
  

	
	  

	Notary Public in and for the State and County/City aforesaid

  

					
	My commission expires:	  	  
	  	
		
	Printed Name of Notary Public:	  	

 TRUSTEE’S CERTIFICATE OF AUTHENTICATION 

This is one of the Securities of a series issued under the Indenture described herein. 

Dated: 
  

			
	 THE BANK OF NEW YORK MELLON TRUST COMPANY, N.A.

as Trustee

		
	By:	 	  

		 	Authorized Officer

 FORM OF TRANSFER NOTICE 

FOR VALUE RECEIVED the undersigned registered holder hereby sell(s), assign(s) and transfer(s) unto 

Insert Taxpayer Identification No. 
  

 
 Please print or typewrite name and address including
zip code of assignee 
  
  

the within Security and all rights thereunder, hereby irrevocably constituting and appointing 

                          
               attorney to transfer said Security on the books of the Security Registrar with full power of substitution in the premises. 

 

							
	Date:	 	  
	 		 	  

		 		 		 	NOTICE: The signature to this assignment must correspond with the name as written upon the face of the within-mentioned instrument in every particular, without alteration or any change
whatsoever.

 EXHIBIT A 

Schedule of ExchangesEX-4.1

 Exhibit 4.1 

REGISTRATION 
 RIGHTS
AGREEMENT 
 dated as of 

[—], 2014 

among 
 AURIS MEDICAL
HOLDING AG 
 and 

THE SHAREHOLDERS PARTY HERETO 

 TABLE OF CONTENTS 

 
  

 

							
	 	 	 	  	PAGE	 
	
	 ARTICLE 1

Definitions
	   
   

			
	 Section 1.01.
	 	 Definitions
	  	 	1	  
	 Section 1.02.
	 	 Other Definitional and Interpretative Provisions
	  	 	4	  
	
	 ARTICLE 2

Registration Rights
	   
   

			
	 Section 2.01.
	 	 Demand Registration
	  	 	5	  
	 Section 2.02.
	 	 Piggyback Registration
	  	 	7	  
	 Section 2.03.
	 	 Shelf Registration
	  	 	8	  
	 Section 2.04.
	 	 Registration Procedures
	  	 	10	  
	 Section 2.05.
	 	 Participation In Public Offering
	  	 	13	  
	 Section 2.06.
	 	 Rule 144 Sales; Cooperation By The Company
	  	 	13	  
	
	 ARTICLE 3

Indemnification and Contribution
	   
   

			
	 Section 3.01.
	 	 Indemnification by the Company
	  	 	14	  
	 Section 3.02.
	 	 Indemnification by Participating Shareholders
	  	 	14	  
	 Section 3.03.
	 	 Conduct of Indemnification Proceedings
	  	 	15	  
	 Section 3.04.
	 	 Contribution
	  	 	15	  
	 Section 3.05.
	 	 Other Indemnification
	  	 	16	  
	
	 ARTICLE 4

Miscellaneous
	   
   

			
	 Section 4.01.
	 	 Binding Effect; Assignability; Benefit
	  	 	16	  
	 Section 4.02.
	 	 Notices
	  	 	17	  
	 Section 4.03.
	 	 Waiver; Amendment; Termination
	  	 	18	  
	 Section 4.04.
	 	 Governing Law
	  	 	18	  
	 Section 4.05.
	 	 Jurisdiction
	  	 	18	  
	 Section 4.06.
	 	 WAIVER OF JURY TRIAL
	  	 	19	  
	 Section 4.07.
	 	 Specific Enforcement
	  	 	19	  
	 Section 4.08.
	 	 Counterparts; Effectiveness
	  	 	19	  
	 Section 4.09.
	 	 Entire Agreement
	  	 	19	  
	 Section 4.10.
	 	 Severability
	  	 	19	  
	 Section 4.11.
	 	 Confidentiality
	  	 	20	  
	 Section 4.12.
	 	 Independent Nature of Shareholders’ Obligations and Rights
	  	 	20	  
			
	 Exhibit A
	 	 Joinder Agreement
	  			

 REGISTRATION RIGHTS AGREEMENT 

THIS REGISTRATION RIGHTS AGREEMENT is made as of [—], 2014 (this
“Agreement”) among AURIS MEDICAL HOLDING AG, a corporation organized under the laws of Switzerland (the “Company”), and the Shareholders party hereto as listed on the signature pages, including any Permitted
Transferees (collectively, the “Shareholders”).  
 In consideration of the mutual promises made herein and other
good and valuable consideration, the receipt and sufficiency of which are hereby acknowledged, the parties hereto agree as follows: 

ARTICLE 1 

DEFINITIONS 

Section 1.01. Definitions. (a) The following terms, as used herein, have the following meanings: 

“Affiliate” means, with respect to any Person, any other Person directly or indirectly controlling, controlled by or
under common control with such Person, provided that no securityholder of the Company shall be deemed an Affiliate of any other securityholder solely by reason of any investment in the Company. For the purpose of this
definition, the term “control” (including, with correlative meanings, the terms “controlling”, “controlled by” and “under common control with”), as used with respect to any Person, shall mean the possession,
directly or indirectly, of the power to direct or cause the direction of the management and policies of such Person, whether through the ownership of voting securities, by contract or otherwise. 

“Business Day” means any day except a Saturday, Sunday or other day on which commercial banks in New York City are
authorized by law to close. 
 “Common Shares” means common shares, nominal value CHF 0.40 per share, of
the Company and any shares into which such Common Shares may thereafter be converted or changed. 
 “Company
Securities” means the Common Shares and, for purposes of the definitions of “Permitted Transferees” and “Transfer”, shall also mean any other securities of the Company that are convertible or exercisable
into or exchangeable for shares of Common Shares. 
 “Exchange Act” means the Securities Exchange Act of 1934, as
amended. 
 “FINRA” means the Financial Industry Regulatory Authority (formerly, the National Association of Securities
Dealers, Inc.) and any successor thereto. 
 “First Public Offering” means the Company’s initial Public Offering. 

 “Permitted Transferee” means in the case of any Shareholder, a Person to
whom Registrable Securities are Transferred by such Shareholder; provided that (i) such Transfer does not violate any agreements between such Shareholder and the Company or any of the Company’s subsidiaries,
(ii) such Transfer is not made in a registered offering or pursuant to Rule 144 and (iii) such transferee shall only be a Permitted Transferee if and to the extent the transferor designates the transferee as a Permitted Transferee entitled
to rights hereunder pursuant to Section 4.01(b). 
 “Person” means an individual, corporation, limited
liability company, partnership, association, trust or other entity or organization, including a government or political subdivision or an agency or instrumentality thereof. 

“Public Offering” means an underwritten public offering of Registrable Securities of the Company pursuant to an
effective registration statement under the Securities Act, other than pursuant to a registration statement on Form S-4, Form F-4 or Form S-8 or any similar or successor form. 

“Registrable Securities” means, at any time, any Company Securities and any other securities issued or issuable by the
Company or any of its successors or assigns in respect of any such Company Securities by way of conversion, exchange, exercise, dividend, split, reverse split, combination, recapitalization, reclassification, merger, amalgamation, consolidation,
sale of assets, other reorganization or otherwise or which by their terms are exercisable or exchangeable for or convertible into Company Securities until (i) a registration statement covering such Company Securities or such other securities
has been declared effective by the SEC and such Company Securities or such other securities have been disposed of pursuant to such effective registration statement, (ii) such Company Securities or such other securities are sold under
circumstances in which all of the applicable conditions of Rule 144 are met or (iii) such Company Securities or such other securities are eligible for sale by the holder thereof under Rule 144 without any limitation thereunder (including with
respect to volume or manner of sale) or need for current public information. 
 “Registration Expenses” means
any and all expenses incident to the performance of, or compliance with, any registration or marketing of securities, including all (i) registration and filing fees, and all other fees and expenses payable in connection with the listing of
securities on any securities exchange or automated interdealer quotation system, (ii) fees and expenses of compliance with any securities or “blue sky” laws (including reasonable fees and disbursements of counsel in connection with
“blue sky” qualifications of the securities registered), (iii) expenses in connection with the preparation, printing, mailing and delivery of any registration statements, prospectuses and other documents in connection therewith and
any amendments or supplements thereto, (iv) security engraving and printing expenses, (v) internal expenses of the Company (including all salaries and expenses of its officers and employees performing legal or accounting duties),
(vi) reasonable fees and disbursements of counsel for the Company and customary fees and expenses for independent certified public accountants retained by the Company (including the expenses relating to any comfort letters or costs associated
with the delivery by independent certified public accountants of any comfort 

  
 2 

 
letters requested pursuant to Section 2.04(h)), (vii) reasonable fees and expenses of any special experts retained by the Company in connection with such registration, (viii) reasonable
and documented fees, out-of-pocket costs and expenses of the Shareholders, including the reasonable fees and disbursements of one counsel for all of the Shareholders participating in the offering selected by the Shareholders holding two-thirds of
the Registrable Securities to be sold for the account of all Shareholders in the offering, (ix) fees and expenses in connection with any review by FINRA of the underwriting arrangements or other terms of the offering, and all fees and expenses
of any “qualified independent underwriter,” including the fees and expenses of any counsel thereto, (x) fees and disbursements of underwriters customarily paid by issuers or sellers of securities, but excluding any underwriting fees,
discounts and commissions attributable to the sale of Registrable Securities, (xi) costs of printing and producing any agreements among underwriters, underwriting agreements, any “blue sky” or legal investment memoranda and any
selling agreements and other documents in connection with the offering, sale or delivery of the Registrable Securities, (xii) transfer agents’ and registrars’ fees and expenses and the fees and expenses of any other agent or trustee
appointed in connection with such offering, and (xiii) expenses relating to any analyst or investor presentations or any “road shows” undertaken in connection with the registration, marketing or selling of the Registrable Securities.
Except as set forth in clause (viii) above, Registration Expenses shall not include any out-of-pocket expenses of the Shareholders (or the agents who manage their accounts). For the avoidance of doubt, Registration Expenses shall not include
any underwriting fees, discounts, commissions or taxes attributable to the sale of Registrable Securities. 
 “Rule 144”
means Rule 144 (or any successor or similar provisions) under the Securities Act. 
 “SEC” means the Securities and
Exchange Commission. 
 “Securities Act” means the Securities Act of 1933, as amended. 

“Shareholder” means at any time, any Person (other than the Company) who shall then be a party to or bound by this Agreement,
so long as such Person shall “beneficially own” (as such term is defined in Rule 13d-3 of the Exchange Act) any Company Securities. 

“Transfer” means, with respect to any Company Securities, (i) when used as a verb, to sell, assign, dispose of,
exchange, pledge, encumber, hypothecate or otherwise transfer such Company Securities or any participation or interest therein, whether directly or indirectly, or agree or commit to do any of the foregoing and (ii) when used as a noun, a direct
or indirect sale, assignment, disposition, exchange, pledge, encumbrance, hypothecation, or other transfer of such Company Securities or any participation or interest therein or any agreement or commitment to do any of the foregoing. 

“Trigger Date” means the date six months after the closing of the First Public Offering. 

  
 3 

 (b) Each of the following terms is defined in the Section set forth opposite such term: 

 

			
	 Term
	  	 Section

	 Agents
	  	4.11
	 Agreement
	  	Preamble
	 Company
	  	Preamble
	 Damages
	  	3.01
	 Demand Registration
	  	2.01(a)
	 Indemnified Party
	  	3.03
	 Indemnifying Party
	  	3.03
	 Inspectors
	  	2.04(g)
	 Joinder Agreement
	  	4.01(b)
	 Lock-Up Period
	  	
	 Maximum Offering Size
	  	2.01(e)
	 Notice
	  	4.02
	 Piggyback Registration
	  	2.02(a)
	 Records
	  	2.04(g)
	 Registering Shareholders
	  	2.01(a)(ii)
	 Requesting Shareholder
	  	2.01(a)
	 Shelf Registration
	  	2.03(a)
	 Shelf Requesting Shareholder
	  	2.03(a)
	 Shelf Registering Shareholder
	  	2.03(b)(ii)
	 Underwritten Takedown
	  	2.03(a)

 Section 1.02. Other Definitional and Interpretative Provisions. The words “hereof”,
“herein” and “hereunder” and words of like import used in this Agreement shall refer to this Agreement as a whole and not to any particular provision of this Agreement. The captions herein are included for convenience of
reference only and shall be ignored in the construction or interpretation hereof. References to Articles, Sections or Exhibits are to Articles, Sections and Exhibits of this Agreement unless otherwise specified. All Exhibits annexed hereto or
referred to herein are hereby incorporated in and made a part of this Agreement as if set forth in full herein. Any capitalized term used in any Exhibit but not otherwise defined therein shall have the meaning as defined in this Agreement. Any
singular term in this Agreement shall be deemed to include the plural, and any plural term the singular. Whenever the words “include”, “includes” or “including” are used in this Agreement, they shall be deemed to be
followed by the words “without limitation”, whether or not they are in fact followed by those words or words of like import. “Writing”, “written” and comparable terms refer to printing, typing and other means of
reproducing words (including electronic media) in a visible form. References to any agreement or contract are to that agreement or contract as amended, modified or supplemented from time to time in accordance with the terms hereof and thereof.
References to any Person include the successors and permitted assigns of that Person. References from or through any date mean, unless otherwise specified, from and including or through and including, respectively. 

  
 4 

 ARTICLE 2 

REGISTRATION RIGHTS 

Section 2.01. Demand Registration. (a) At any time after the Trigger Date, if the Company shall receive a request from a
Shareholder or group of Shareholders, in each case holding at least two-thirds of the Registrable Securities (the requesting Shareholder(s) shall be referred to herein as the “Requesting Shareholder”) that the Company effect the
registration under the Securities Act of all or any portion of the Requesting Shareholder’s Registrable Securities, and specifying the intended method of disposition thereof, then the Company shall as promptly as practicable following the date
of receipt by the Company of such request give notice of such requested registration (each such request shall be referred to herein as a “Demand Registration”) at least 10 Business Days prior to the anticipated filing date of the
registration statement relating to such Demand Registration to the other Shareholders and thereupon shall use its commercially reasonable efforts to effect, as expeditiously as possible, the registration under the Securities Act of: 

(i) subject to the restrictions set forth in Sections 2.01(e), all Registrable Securities for which the Requesting Shareholder
has requested registration under this Section 2.01, and 
 (ii) subject to the restrictions set forth in Sections 2.01(e) and
2.02, all other Registrable Securities of the same class as those requested to be registered by the Requesting Shareholder that any Shareholders (all such Shareholders, together with the Requesting Shareholder, the “Registering
Shareholders”) have requested the Company to register pursuant to Section 2.02, by request received by the Company within five Business Days after such Shareholders receive the Company’s notice of the Demand Registration, 

all to the extent necessary to permit the disposition (in accordance with the intended methods thereof as aforesaid) of the Registrable Securities so
to be registered, provided that the Company shall not be obligated to effect a Demand Registration unless the aggregate proceeds expected to be received from the sale of the Registrable Securities requested to be included in such Demand
Registration equals or exceeds $10,000,000 or such lesser amount that constitutes all of the Requesting Shareholder’s Registrable Securities (provided that such lesser amount is at least $5,000,000). In no event shall the Company be required to
effect more than two Demand Registrations or Underwritten Takedowns hereunder. 
 (b) Promptly after the expiration of the
five-Business Day period referred to in Section 2.01(a)(ii), the Company will notify all Registering Shareholders of the identities of the other Registering Shareholders and the number of shares of Registrable Securities requested to be included
therein. At any time prior to the effective date of the registration statement relating to such registration, the Requesting Shareholder may revoke such 

  
 5 

 
request, without liability to any of the other Registering Shareholders, by providing a notice to the Company revoking such request. A request, so revoked, shall be considered to be a Demand
Registration unless (i) such revocation arose out of the fault of the Company (in which case the Company shall be obligated to pay all Registration Expenses in connection with such revoked request), including without limitation if the
Requesting Shareholders shall have learned of a material adverse change in the condition, business, or prospects of the Company from that known to the Requesting Shareholder at the time of its request and have withdrawn the request with reasonable
promptness after learning of such information,or (ii) the Requesting Shareholder reimburses the Company for all Registration Expenses of such revoked request. 

(c) The Company shall be liable for and shall pay all Registration Expenses in connection with any Demand Registration, regardless of whether
such registration is effected, unless the Requesting Shareholder elects to pay such Registration Expenses as described in the last sentence of Section 2.01(b). 

(d) A Demand Registration shall not be deemed to have occurred: 

(i) unless the registration statement relating thereto (A) has become effective under the Securities Act and (B) has
remained effective for a period of at least 180 days (or such shorter period in which all Registrable Securities of the Registering Shareholders included in such registration have actually been sold thereunder), provided that a Demand
Registration shall not be deemed to have occurred if, after such registration statement becomes effective, (1) such registration statement is interfered with by any stop order, injunction or other order or requirement of the SEC or other
governmental agency or court and (2) less than 75% of the Registrable Securities included in such registration statement have been sold thereunder; or 

(ii) if the Maximum Offering Size is reduced in accordance with Section 2.01(e) such that less than 66-2/3% of the Registrable
Securities of the Requesting Shareholders sought to be included in such registration are included. 
 (e) If a Demand Registration involves
an underwritten Public Offering and the managing underwriter advises the Company and the Requesting Shareholder that, in its view, the number of shares of Registrable Securities requested to be included in such registration (including any securities
that the Company proposes to be included that are not Registrable Securities) exceeds the largest number of shares that can be sold without having an adverse effect on such offering, including the price at which such shares can be sold (the
“Maximum Offering Size”), the Company shall include in such registration, in the priority listed below, up to the Maximum Offering Size: 

(i) first, all Registrable Securities requested to be included in such registration by all Registering Shareholders (allocated,
if necessary for the offering not to exceed the Maximum Offering Size, pro rata among such Shareholders on the basis of the relative number of Registrable Securities held by each such Shareholder); and 

  
 6 

 (ii) second, any securities proposed to be registered by the Company (including
for the benefit of any other Persons not party to this Agreement). 
 (f) Upon notice to the Requesting Shareholder, the Company may
postpone effecting a registration pursuant to this Section 2.01 on two occasions during any period of twelve consecutive months for a reasonable time specified in the notice but not exceeding 90 days (which period may not be extended or renewed), if
(i) the Company reasonably determines that effecting the registration would materially and adversely affect an offering of securities of the Company the preparation of which had then been commenced, or (ii) the Company is in possession of
material non-public information the disclosure of which during the period specified in such notice the Company reasonably believes would not be in the best interests of the Company. 

Section 2.02. Piggyback Registration. (a) At any time after the Trigger Date, if the Company proposes to register any Company
Securities under the Securities Act (other than (i) a Shelf Registration, which will be subject to the provisions of Section 2.03; provided that any Underwritten Takedown will be subject to this Section 2.02, or (ii) a registration on Form
S-8, F-4 or S-4, or any successor or similar form, whether or not for sale for its own account, the Company shall each such time give prompt notice at least two Business Days prior to the anticipated filing date of the registration statement
relating to such registration to each Shareholder, which notice shall set forth such Shareholder’s rights under this Section 2.02 and shall offer such Shareholder the opportunity to include in such registration statement the number of
Registrable Securities of the same class or series as those proposed to be registered as each such Shareholder may request (a “Piggyback Registration”), subject to the provisions of Section 2.02(b). Upon the request of any such
Shareholder made within two Business Days after the receipt of notice from the Company (which request shall specify the number of Registrable Securities intended to be registered by such Shareholder), the Company shall use all commercially
reasonable efforts to effect the registration under the Securities Act of all Registrable Securities that the Company has been so requested to register by all such Shareholders, to the extent required to permit the disposition of the Registrable
Securities so to be registered, provided that (A) if such registration involves an underwritten Public Offering, all such Shareholders requesting to be included in the Company’s registration must sell their Registrable Securities to
the underwriters selected as provided in Section 2.04(f) on the same terms and conditions as apply to the Company or the Requesting Shareholders, as applicable, and (B) if, at any time after giving notice of its intention to register any
Company Securities pursuant to this Section 2.02(a) and prior to the effective date of the registration statement filed in connection with such registration, the Company shall determine for any reason not to register such securities, the Company
shall give notice to all such Shareholders and, thereupon, shall be relieved of its obligation to register any Registrable Securities in connection with such registration. No registration effected under this Section 2.02 shall relieve the Company of
its obligations to effect a Demand Registration to the extent required by Section 2.01 or a Shelf Registration to the extent required by Section 2.03. The Company shall pay all Registration Expenses in connection with each Piggyback Registration.

  
 7 

 (b) If a Piggyback Registration involves an underwritten Public Offering (other than any Demand
Registration, in which case the provisions with respect to priority of inclusion in such offering set forth in Section 2.01(e) shall apply) and the managing underwriter advises the Company that, in its view, the number of Shares that the Company and
such Shareholders intend to include in such registration exceeds the Maximum Offering Size, the Company shall include in such registration, in the following priority, up to the Maximum Offering Size: 

(i) first, so much of the Company Securities proposed to be registered for the account of the Company (or, if such registration
is pursuant to a demand by a Person that is not a Shareholder, for the account of such other Person) as would not cause the offering to exceed the Maximum Offering Size, 

(ii) second, all Registrable Securities requested to be included in such registration by any Shareholders pursuant to this
Section 2.02 (allocated, if necessary for the offering not to exceed the Maximum Offering Size, pro rata among such Shareholders on the basis of the relative number of shares of Registrable Securities so requested to be included in such registration
by each), and 
 (iii) third, any securities proposed to be registered for the account of any other Persons with such
priorities among them as the Company shall determine. 
 Section 2.03. Shelf Registration. (a) At any time after the first
anniversary of the First Public Offering, if the Company is eligible to use Form F-3 or Form S-3, a Shareholder or group of Shareholders, in each case holding at least two-thirds of the Registrable Securities (the requesting Shareholder(s) shall be
referred to herein as the “Shelf Requesting Shareholder”) may request the Company to effect a registration of some or all of the Registrable Securities held by such Shelf Requesting Shareholder under a Registration Statement
pursuant to Rule 415 under the Securities Act (or any successor or similar rule) (a “Shelf Registration”). The Company shall only be required to effectuate one Public Offering from such Shelf Registration (an “Underwritten
Takedown”) within any six-month period, which offering shall be deemed a Demand Registration. The provisions of Section 2.01 shall apply mutatis mutandis to each Underwritten Takedown except as otherwise provided in this Section 2.03, with
references to “filing of the registration statement” or “effective date” being deemed references to filing of a prospectus or supplement for such offering, references to “registration” being deemed
references to the offering, references to “Demand Registration” being deemd references to “Shelf Registration” or “Underwritten Takedown,” as applicable, and references to “Registering
Shareholders” being deemed references to “Shelf Registering Shareholders”; provided that Shelf Registering Shareholders shall only include Shareholders whose Registrable Securities are included in such Shelf Registration or
may be included therein without the need for an amendment to such Shelf Registration (other than an automatically effective amendment). So long as the Shelf Registration is effective, no Shareholder may request any Demand Registration pursuant to
Section 2.01 with respect to Registrable Shares that are registered or registrable without the need for an amendment (other than an automatically effective amendment) on such Shelf Registration. 

  
 8 

 (b) If the Company shall receive a request from a Shelf Requesting Shareholder that the Company
effect a Shelf Registration, then the Company shall as promptly as practicable following the date of receipt by the Company of such request give notice of such requested registration at least ten Business Days prior to the anticipated filing date of
the registration statement relating to such Shelf Registration to the other Shareholders and thereupon shall use its reasonable best efforts to effect, as expeditiously as possible, the registration under the Securities Act of: 

(i) all Registrable Securities for which the Shelf Requesting Shareholder has requested registration under this Section 2.03,
and 
 (ii) all other Registrable Securities of the same class as those requested to be registered by the Shelf Requesting
Shareholder that any Shareholders (all such Shareholders, together with the Shelf Requesting Shareholder, the “Shelf Registering Shareholders”) have requested the Company to register by request received by the Company within two
Business Days after such Shareholders receive the Company’s notice of the Shelf Registration, 
 all to the extent necessary to permit the registration
of the Registrable Securities so to be registered on such Shelf Registration. 
 (c) At any time prior to the effective date of the
registration statement relating to such Shelf Registration, the Shelf Requesting Shareholder may revoke such request, without liability to any of the other Shelf Registering Shareholders, by providing a notice to the Company revoking such request.

 (d) The Company shall be liable for and pay all Registration Expenses in connection with any Shelf Registration. 

(e) Upon notice to the Shelf Requesting Shareholder, the Company may postpone effecting a registration pursuant to this Section 2.03 on one
occasion during any period of six consecutive months for a reasonable time specified in the notice but not exceeding 90 days (which period may not be extended or renewed), if the Company determines that effecting the registration would materially
and adversely affect an offering of securities of the Company the preparation of which had then been commenced, or the Company is in possession of material non-public information the disclosure of which during the period specified in such notice the
Company reasonably believes would not be in the best interests of the Company. 

  
 9 

 Section 2.04. Registration Procedures. Whenever Shareholders request that any
Registrable Securities be registered pursuant to Section 2.01 or 2.02, or the Company prepares a Shelf Registration pursuant to Section 2.03, subject to the provisions of such Sections, the Company shall use all commercially reasonable efforts to
effect the registration and the sale of such Registrable Securities in accordance with the intended method of disposition thereof as quickly as practicable, and, in connection with any such request: 

(a) The Company shall as expeditiously as possible prepare and file with the SEC a registration statement on any form for which the Company
then qualifies or that counsel for the Company shall deem appropriate and which form shall be available for the sale of the Registrable Securities to be registered thereunder in accordance with the intended method of distribution thereof, and use
all commercially reasonable efforts to cause such filed registration statement to become and remain effective for a period of not less than 180 days, or in the case of a Shelf Registration, three years (or such shorter period in which all of the
Registrable Securities of the Shareholders included in such registration statement shall have actually been sold thereunder or cease to be Registrable Securities). Any such registration statement shall be an automatically effective registration
statement to the extent permitted by the SEC’s rules and regulations. 
 (b) Prior to filing a registration statement or prospectus or
any amendment or supplement thereto (other than any report filed pursuant to the Exchange Act that is incorporated by reference therein), the Company shall, if requested, furnish to each participating Shareholder and each underwriter, if any, of the
Registrable Securities covered by such registration statement copies of such registration statement as proposed to be filed, and thereafter the Company shall furnish to such Shareholder and underwriter, if any, such number of copies of such
registration statement, each amendment and supplement thereto (in each case including all exhibits thereto and documents incorporated by reference therein), the prospectus included in such registration statement (including each preliminary
prospectus and any summary prospectus) and any other prospectus filed under Rule 424, Rule 430A, Rule 430B or Rule 430C under the Securities Act and such other documents as such Shareholder or underwriter may reasonably request in order to
facilitate the disposition of the Registrable Securities owned by such Shareholder. 
 (c) After the filing of the registration statement,
the Company shall (i) cause the related prospectus to be supplemented by any required prospectus supplement and, as so supplemented, to be filed pursuant to Rule 424 under the Securities Act, (ii) comply with the provisions of the
Securities Act with respect to the disposition of all Registrable Securities covered by such registration statement during the applicable period in accordance with the intended methods of disposition by the Shareholders thereof set forth in such
registration statement or supplement to such prospectus and (iii) promptly notify each Shareholder holding Registrable Securities covered by such registration statement of any stop order issued or threatened by the SEC or any state securities
commission and take all reasonable actions required to prevent the entry of such stop order or to remove it if entered. 
 (d) The Company
shall use all commercially reasonable efforts to (i) register or qualify the Registrable Securities covered by such registration statement under such other securities or “blue sky” laws of such jurisdictions in the United
States as any Registering Shareholder holding such Registrable Securities reasonably (in light of such Shareholder’s intended plan of distribution) requests and (ii) cause such Registrable 

  
 10 

 
Securities to be registered with or approved by such other governmental agencies or authorities as may be necessary by virtue of the business and operations of the Company and do any and all
other acts and things that may be reasonably necessary or advisable to enable such Shareholder to consummate the disposition of the Registrable Securities owned by such Shareholder, provided that the Company shall not be required to
(A) qualify generally to do business in any jurisdiction where it would not otherwise be required to qualify but for this Section 2.04(d), (B) subject itself to taxation in any such jurisdiction or (C) consent to general service of process
in any such jurisdiction. 
 (e) The Company shall immediately notify each Shareholder holding such Registrable Securities covered by such
registration statement, at any time when a prospectus relating thereto is required to be delivered under the Securities Act, of the occurrence of an event requiring the preparation of a supplement or amendment to such prospectus so that, as
thereafter delivered to the purchasers of such Registrable Securities, such prospectus will not contain an untrue statement of a material fact or omit to state any material fact required to be stated therein or necessary to make the statements
therein not misleading and promptly prepare and make available to each such Shareholder and file with the SEC any such supplement or amendment. 

(f) The Company shall have the right to select an underwriter or underwriters in connection with any Public Offering resulting from any
exercise of a Demand Registration (including any Underwritten Takedown), which underwriter or underwriters shall be reasonably acceptable to the Requesting Shareholder. In connection with any Public Offering, the Company shall enter into customary
agreements (including an underwriting agreement in customary form) and take such all other actions as are reasonably required in order to expedite or facilitate the disposition of such Registrable Securities in any such Public Offering, including
the engagement of a “qualified independent underwriter” in connection with the qualification of the underwriting arrangements with FINRA. 

(g) Upon execution of confidentiality agreements in form and substance reasonably satisfactory to the Company, the Company shall, in
connection with a Public Offering make available for inspection by any Shareholder and any underwriter participating in any disposition pursuant to a registration statement being filed by the Company pursuant to this Section 2.04 and any attorney,
accountant or other professional retained by any such Shareholder or underwriter (collectively, the “Inspectors”), all financial and other records, pertinent corporate documents and properties of the Company (collectively, the
“Records”) as shall be reasonably necessary or desirable to enable any of the Inspectors to exercise its due diligence responsibility, and cause the Company’s officers, directors and employees to supply all information
reasonably requested by any Inspectors in connection with such registration statement. Records that the Company determines, in good faith, to be confidential and that it notifies the Inspectors are confidential shall not be disclosed by the
Inspectors unless (i) the disclosure of such Records is necessary to avoid or correct a material misstatement or omission in such registration statement or (ii) the release of such Records is ordered pursuant to a subpoena or other order
from a court of competent jurisdiction. Each Shareholder agrees that information obtained by it as a result of such inspections shall be deemed 

  
 11 

 
confidential and shall not be used by it or its Affiliates as the basis for any market transactions in the Company Securities unless and until such information is made generally available to the
public. Each Shareholder further agrees that, upon learning that disclosure of such Records is sought in a court of competent jurisdiction, it shall give notice to the Company and allow the Company, at its expense, to undertake appropriate action to
prevent disclosure of the Records deemed confidential. 
 (h) In connection with any Public Offering, the Company shall use commercially
reasonable efforts to furnish to each Registering Shareholder and to each such underwriter, if any, a signed counterpart, addressed to such Shareholder or underwriter, of (i) an opinion or opinions of counsel to the Company and (ii) a
comfort letter or comfort letters from the Company’s independent public accountants, each in customary form and covering such matters of the kind customarily covered by opinions or comfort letters, as the case may be, as the managing
underwriter therefor reasonably requests. 
 (i) The Company shall otherwise use all commercially reasonable efforts to comply with all
applicable rules and regulations of the SEC, and make available to its security holders, as soon as reasonably practicable, an earnings statement or such other document covering a period of 12 months, beginning within three months after the
effective date of the registration statement, which earnings statement satisfies the requirements of Rule 158 under the Securities Act. 

(j) The Company may require each Shareholder promptly to furnish in writing to the Company such information regarding the distribution of the
Registrable Securities as the Company may from time to time reasonably request and such other information as may be legally required in connection with such registration. In connection with a Shelf Registration, any Shareholder that does not provide
such information within two Business Days of a request by the Company (which request is made before filing of the Shelf Registration) may have its Registrable Securities excluded from such Shelf Registration; provided that such securities shall be
added within fifteen Business Days after the Shareholder provides such information if the Company may add such securities to such Shelf Registration without the need for a post-effective amendment (other than an automatically effective amendment) to
the Shelf Registration. 
 (k) Each Shareholder agrees that, upon receipt of any notice from the Company of the happening of any event of
the kind described in Section 2.04(e), such Shareholder shall forthwith discontinue disposition of Registrable Securities pursuant to the registration statement covering such Registrable Securities until such Shareholder’s receipt of the copies
of the supplemented or amended prospectus contemplated by Section 2.04(e), and, if so directed by the Company, such Shareholder shall deliver to the Company all copies, other than any permanent file copies then in such Shareholder’s possession,
of the most recent prospectus covering such Registrable Securities at the time of receipt of such notice. If the Company shall give such notice, the Company shall extend the period during which such registration statement shall be maintained
effective (including the period referred to in Section 2.04(a)) by the number of days during the period from and including the date of the giving of notice pursuant to Section 2.04(e) to the date when the Company shall make available to such
Shareholder a prospectus supplemented or amended to conform with the requirements of Section 2.04(e). 

  
 12 

 (l) The Company shall use all commercially reasonable efforts to list all Registrable Securities
covered by such registration statement on any securities exchange or quotation system on which the Common Shares are then listed or traded. 

(m) In any Public Offering pursuant to a Demand Registration, the Company shall have appropriate officers of the Company (i) prepare and
make presentations at any “road shows” and before analysts and (ii) otherwise use their reasonable efforts to cooperate as reasonably requested by the underwriters in the offering, marketing or selling of the Registrable Securities.

 (n) Each Shareholder agrees that, in connection with any offering pursuant to this Agreement, it will not prepare or use or refer to, any
“free writing prospectus” (as defined in Rule 405 of the Securities Act) without the prior written authorization of the Company (which authorization shall not be unreasonably withheld), and will not distribute any written materials in
connection with the offer or sale of the Registrable Securities pursuant to any registration statement hereunder other than the Prospectus and any such free writing prospectus so authorized. 

Section 2.05. Participation In Public Offering. No Shareholder may participate in any Public Offering hereunder unless such
Shareholder (a) agrees to sell such Shareholder’s Registrable Securities on the basis provided in any underwriting arrangements approved by the Persons entitled hereunder to approve such arrangements and (b) completes and executes all
questionnaires, powers of attorney, indemnities, underwriting agreements and other documents reasonably required under the terms of such underwriting arrangements and the provisions of this Agreement in respect of registration rights. 

Section 2.06. Rule 144 Sales; Cooperation By The Company. If any Shareholder shall transfer any Registrable Securities pursuant to
Rule 144, the Company shall cooperate, to the extent commercially reasonable, with such Shareholder and shall provide to such Shareholder such information as such Shareholder shall reasonably request. Without limiting the foregoing, the Company
shall at any time after any of the Company’s shares are registered under the Securities Act or the Exchange Act: (i) make and keep available public information, as those terms are contemplated by Rule 144; (ii) timely file with the
SEC all reports and other documents required to be filed under the Securities Act and the Exchange Act; and (iii) furnish to each Shareholder forthwith upon request a written statement by the Company as to its compliance with the reporting
requirements of the Securities Act and the Exchange Act, a copy of the most recent annual or quarterly report of the Company, and such other information as such Shareholder may reasonably request in order to avail itself of any rule or regulation of
the SEC allowing such Shareholder to sell any Registrable Securities without registration. 

  
 13 

 ARTICLE 3 

INDEMNIFICATION AND CONTRIBUTION 

Section 3.01. Indemnification by the Company. The Company agrees to indemnify and hold harmless each Shareholder beneficially
owning any Registrable Securities covered by a registration statement, its officers, directors, employees, partners and agents, and each Person, if any, who controls such Shareholder within the meaning of Section 15 of the Securities Act or
Section 20 of the Exchange Act from and against any and all losses, claims, damages, liabilities and expenses (including reasonable expenses of investigation and reasonable attorneys’ fees and expenses) (collectively,
“Damages”) caused by or relating to any untrue statement or alleged untrue statement of a material fact contained in any registration statement or prospectus relating to the Registrable Securities (as amended or supplemented if the
Company shall have furnished any amendments or supplements thereto) or any preliminary prospectus or free-writing prospectus (as defined in Rule 405 under the Securities Act), or caused by or relating to any omission or alleged omission to state
therein a material fact required to be stated therein or necessary to make the statements therein not misleading, except insofar as such Damages are caused by or related to any such untrue statement or omission or alleged untrue statement or
omission so made based upon information furnished in writing to the Company by such Shareholder or on such Shareholder’s behalf expressly for use therein. The Company also agrees to indemnify any underwriters of the Registrable Securities,
their officers and directors and each Person who controls such underwriters within the meaning of Section 15 of the Securities Act or Section 20 of the Exchange Act on substantially the same basis as that of the indemnification of the
Shareholders provided in this Section 3.01. 
 Section 3.02. Indemnification by Participating Shareholders. Each Shareholder
holding Registrable Securities included in any registration statement agrees, severally but not jointly, to indemnify and hold harmless the Company, its officers, directors and agents and each Person, if any, who controls the Company within the
meaning of Section 15 of the Securities Act or Section 20 of the Exchange Act to the same extent as the indemnity from the Company to such Shareholder provided in Section 3.01, but only with respect to information furnished in writing by
such Shareholder or on such Shareholder’s behalf expressly for use in any registration statement or prospectus relating to the Registrable Securities, or any amendment or supplement thereto, or any preliminary prospectus or free-writing
prospectus. Each such Shareholder also agrees to indemnify and hold harmless underwriters of the Registrable Securities, their officers and directors and each Person who controls such underwriters within the meaning of Section 15 of the
Securities Act or Section 20 of the Exchange Act on substantially the same basis as that of the indemnification of the Company provided in this Section 3.02. As a condition to including Registrable Securities in any registration statement filed
in accordance with Article 2, the Company may require that it shall have received an undertaking reasonably satisfactory to it from any underwriter to indemnify and hold it harmless to the extent customarily provided by underwriters with respect to
similar securities. No Shareholder shall be liable under this Section 3.02 for any Damages in excess of, together with any contribution under Section 3.04 below, the net proceeds realized by such Shareholder in the sale of Registrable Securities of
such Shareholder to which such Damages relate. 

  
 14 

 Section 3.03. Conduct of Indemnification Proceedings. If any proceeding (including
any governmental investigation) shall be brought or asserted against any Person in respect of which indemnity may be sought pursuant to this Article 3, such Person (an “Indemnified Party”) shall promptly notify the Person against
whom such indemnity may be sought (the “Indemnifying Party”) in writing and the Indemnifying Party shall assume the defense thereof, including the employment of counsel reasonably satisfactory to such Indemnified Party, and shall
assume the payment of all fees and expenses, provided that the failure of any Indemnified Party so to notify the Indemnifying Party shall not relieve the Indemnifying Party of its obligations hereunder except to the extent that the
Indemnifying Party is materially prejudiced by such failure to notify. In any such proceeding, any Indemnified Party shall have the right to retain its own counsel, but the fees and expenses of such counsel shall be at the expense of such
Indemnified Party unless (a) the Indemnifying Party and the Indemnified Party shall have mutually agreed to the retention of such counsel, (b) in the reasonable judgment of such Indemnified Party representation of both parties by the same
counsel would be inappropriate due to actual or potential differing interests between them, including one or more defenses or counterclaims that are different from or in addition to those available to the Indemnifying Party, or (c) the
Indemnifying Party shall have failed to assume the defense within 60 days of notice pursuant to this Section 3.03. It is understood that, in connection with any proceeding or related proceedings in the same jurisdiction, the Indemnifying Party shall
not be liable for the reasonable fees and expenses of more than one separate firm of attorneys (in addition to one local counsel per jurisdiction) at any time for all such Indemnified Parties, and that all such fees and expenses shall be reimbursed
as they are incurred. In the case of any such separate firm for the Indemnified Parties, such firm shall be designated in writing by the Indemnified Parties. The Indemnifying Party shall not be liable for any settlement of any proceeding effected
without its written consent, but if settled with such consent, or if there be a final judgment for the plaintiff, the Indemnifying Party shall indemnify and hold harmless such Indemnified Parties from and against any loss or liability (to the extent
stated above) by reason of such settlement or judgment. Without the prior written consent of the Indemnified Party, no Indemnifying Party shall effect any settlement of any pending or threatened proceeding in respect of which any Indemnified Party
is or could have been a party and indemnity could have been sought hereunder by such Indemnified Party, unless such settlement (A) includes an unconditional release of such Indemnified Party from all liability arising out of such proceeding,
and (B) does not include any injunctive or other equitable or non-monetary relief applicable to or affecting such Indemnified Person. 

Section 3.04. Contribution. If the indemnification provided for in this Article 3 is unavailable to the Indemnified Parties in
respect of any Damages, then each Indemnifying Party, in lieu of indemnifying the Indemnified Parties, shall contribute to the amount paid or payable by such Indemnified Party, in such proportion as is appropriate to reflect the relative fault of
the Indemnifying Party and Indemnified Party in connection with the actions, statements or omissions that resulted in such Damages as well as any other relevant equitable considerations. The relative fault of such

  
 15 

 
Indemnifying Party and Indemnified Party shall be determined by reference to, among other things, whether any action in question, including any untrue or alleged untrue statement of a material
fact or omission or alleged omission of a material fact, has been taken or made by, or relates to information supplied by, such Indemnifying Party or Indemnified Party, and the parties’ relative intent, knowledge, access to information and
opportunity to correct or prevent such action, statement or omission. The amount paid or payable by a party as a result of any Damages shall be deemed to include, subject to the limitations set forth in this Agreement, any reasonable attorneys’
or other reasonable fees or expenses incurred by such party in connection with any proceeding to the extent such party would have been indemnified for such fees or expenses if the indemnification provided for in this Article 3 was available to such
party in accordance with its terms. 
 The parties hereto agree that it would not be just and equitable if contribution pursuant to this
Section 3.04 were determined by pro rata allocation or by any other method of allocation that does not take into account the equitable considerations referred to in the immediately preceding paragraph. Notwithstanding the provisions of this Section
3.04, no Shareholder shall be required to contribute, in the aggregate, any amount in excess of the amount by which the proceeds actually received by such Shareholder from the sale of the Registrable Securities subject to the proceeding exceeds the
amount of any damages that such Shareholder has otherwise been required to pay by reason of such untrue or alleged untrue statement or omission or alleged omission, except in the case of fraud by such Shareholder. Each Shareholder’s obligation
to contribute pursuant to this Section 3.03 is several in the proportion that the proceeds of the offering received by such Shareholder bears to the total proceeds of the offering received by all such Shareholders and not joint. 

No Person guilty of fraudulent misrepresentation (within the meaning of Section 11(f) of the Securities Act) shall be entitled to
contribution from any Person who was not guilty of such fraudulent misrepresentation. The indemnity and contribution agreements contained in this Article 3 are in addition to any liability that the Indemnifying Parties may have to the Indemnified
Parties. 
 Section 3.05. Other Indemnification. Indemnification similar to that provided in this Article 3 (with appropriate
modifications) shall be given by the Company and each Shareholder participating therein with respect to any required registration or other qualification of securities under any foreign, federal or state law or regulation or governmental authority
other than the Securities Act. 
 ARTICLE 4 

MISCELLANEOUS 

Section 4.01. Binding Effect; Assignability; Benefit. (a) This Agreement shall inure to the benefit of and be binding upon
the parties hereto and their respective heirs, successors, legal representatives and permitted assigns. Any Shareholder that ceases to own beneficially any Registrable Securities shall cease to be bound by the terms hereof (other than (i) the
provisions of Article 3 applicable to such Shareholder with respect to any offering of Registrable Securities completed before the date such Shareholder ceased to own any Registrable Securities and (ii) this Article 4). 

  
 16 

 (b) Neither this Agreement nor any right, remedy, obligation or liability arising hereunder or by
reason hereof shall be assignable by any party hereto pursuant to any Transfer of Registrable Securities or otherwise, except that each Shareholder may assign all or any portion of its rights hereunder to any Permitted Transferee of such Shareholder
with respect to not less than 5% of the outstanding Company Securities at the time of such transfer; provided, however, that no such minimum share assignment requirement shall be necessary for an assignment by a Shareholder which is a
(i) partnership to its partners in accordance with their partnership interests, (ii) a limited liability company to its members in accordance with their interests in the limited liability company, (iii) a corporation to its
stockholders in accordance with their interests in the corporation or (iv) to an Affiliate of such Shareholder. Any such Permitted Transferee shall (unless already bound hereby) execute and deliver to the Company an agreement to be bound by
this Agreement in the form of Exhibit A hereto (a “Joinder Agreement”) and shall thenceforth be a “Shareholder”. 

(c) Nothing in this Agreement, expressed or implied, is intended to confer on any Person other than the parties hereto, and their respective
heirs, successors, legal representatives and permitted assigns, any rights, remedies, obligations or liabilities under or by reason of this Agreement. 

Section 4.02. Notices. All notices, requests and other communications (each, a “Notice”) to any party shall be in
writing and shall be delivered in person, mailed by certified or registered mail, return receipt requested, or sent by facsimile transmission or email transmission so long as receipt of such email is requested and received, 

if to the Company to: 
 Auris
Medical Holding AG 
 Bahnhofstrasse 21 

6300 Zug, Switzerland 
 Fax: +41
61 201 13 51 
 Attention: Sven Zimmermann, Chief Financial Officer 

Email: sz@aurismedical.com 
 with
a copy to: 
 Davis Polk & Wardwell LLP 

450 Lexington Avenue 
 New York,
New York 10017 
 Attention: Richard D. Truesdell, Jr. 

Fax: (212) 701-5674 
 Email:
richard.truesdell@davispolk.com 

  
 17 

 if to any Shareholder, at the address for such Shareholder listed on the signature pages below or
otherwise provided to the Company as set forth below. 
 Any Notice shall be deemed received on the date of receipt by the recipient thereof
if received prior to 5:00 p.m. in the place of receipt and such day is a Business Day in the place of receipt. Otherwise, such Notice shall be deemed not to have been received until the next succeeding Business Day in the place of receipt. Any
Notice sent by facsimile transmission also shall be confirmed by certified or registered mail, return receipt requested, posted within one Business Day after the date of the sending of such facsimile transmission, or by personal delivery, whether
courier or otherwise, made within two Business Days after the date of such facsimile transmission. 
 Any Person that becomes a Shareholder
after the date hereof shall provide its address, fax number and email address to the Company. 
 Section 4.03. Waiver; Amendment;
Termination. (a) The provisions of this Agreement, including the provisions of this sentence, may not be amended, modified or supplemented, and waivers or consents to departures from the provisions hereof may not be given without the
written consent of the Company and holders of two-thirds of the Registrable Securities; provided, however, that in no event shall the obligations of any holder of Registrable Securities be materially increased or the rights of any
Stockholder be adversely affected (without similarly adversely affecting the rights of all Stockholders), except upon the written consent of such holder. Notwithstanding the foregoing, a waiver or consent to depart from the provisions hereof with
respect to a matter that relates exclusively to the rights of holders of Registrable Securities whose securities are being sold pursuant to a Registration Statement and that does not directly or indirectly affect the rights of other holders of
Registrable Securities may be given by holders of at least two-thirds of the Registrable Securities being sold by such holders pursuant to such Registration Statement. 

(b) The right of any Shareholder to request registration or inclusion of Registrable Securities in any registration hereunder shall terminate
upon the earlier to occur of (i) an Exit Event (as defined in the Company’s Shareholders’ Agreement dated as of April 2013, as amended), (ii) the fifth anniversary of the First Public Offering and (iii) the date on which
there are no Registrable Securities. 
 Section 4.04. Governing Law. This Agreement shall be governed by, and construed and
enforced in accordance with, the laws of the State of New York, without regard to the conflicts of laws rules of such state. 

Section 4.05. Jurisdiction. The parties hereby agree that any suit, action or proceeding seeking to enforce any provision of, or
based on any matter arising out of or in connection with, this Agreement or the transactions contemplated hereby shall be brought in any state or federal court in The City of New York, Borough of Manhattan, so long as one of such courts shall have
subject matter jurisdiction over such suit, action or proceeding, and that any cause of action arising out of this Agreement shall be deemed to have arisen from a transaction of business in the State of New York, and each of the

  
 18 

 
parties hereby irrevocably consents to the jurisdiction of such courts (and of the appropriate appellate courts therefrom) in any such suit, action or proceeding and irrevocably waives, to the
fullest extent permitted by law, any objection that it may now or hereafter have to the laying of the venue of any such suit, action or proceeding in any such court or that any such suit, action or proceeding which is brought in any such court has
been brought in an inconvenient forum. Process in any such suit, action or proceeding may be served on any party anywhere in the world, whether within or without the jurisdiction of any such court. Without limiting the foregoing, each party agrees
that service of process on such party as provided in Section 4.02 shall be deemed effective service of process on such party. 

Section 4.06. WAIVER OF JURY TRIAL. EACH OF THE PARTIES HERETO HEREBY IRREVOCABLY WAIVES ANY AND ALL RIGHT TO TRIAL BY JURY IN ANY
LEGAL PROCEEDING ARISING OUT OF OR RELATED TO THIS AGREEMENT OR THE TRANSACTIONS CONTEMPLATED HEREBY. 
 Section 4.07. Specific
Enforcement. Each party hereto acknowledges that the remedies at law of the other parties for a breach or threatened breach of this Agreement would be inadequate and, in recognition of this fact, any party to this Agreement, without posting any
bond or furnishing other security, and in addition to all other remedies that may be available, shall be entitled to obtain equitable relief in the form of specific performance, a temporary restraining order, a temporary or permanent injunction or
any other equitable remedy that may then be available. 
 Section 4.08. Counterparts; Effectiveness. This Agreement may be
executed (including by facsimile or other electronic image scan transmission) with counterpart signature pages or in any number of counterparts, each of which shall be deemed to be an original, and all of which shall, taken together, be considered
one and the same agreement, it being understood that each party need not sign the same counterpart. This Agreement shall become effective when each party hereto shall have executed and delivered this Agreement. Until and unless each party has
executed and delivered this Agreement, this Agreement shall have no effect and no party shall have any right or obligation hereunder (whether by virtue of any other oral or written agreement or other communication). 

Section 4.09. Entire Agreement. This Agreement constitutes the entire agreement and understanding among the parties hereto with
respect to the subject matter of this Agreement and supersedes all prior and contemporaneous agreements and understandings, both oral and written, among the parties hereto with respect to the subject matter hereof. 

Section 4.10. Severability. If any term, provision, covenant or restriction of this Agreement is held by a court of competent
jurisdiction or other authority to be invalid, void or unenforceable, the remainder of the terms, provisions, covenants and restrictions of this Agreement shall remain in full force and effect and shall in no way be affected, impaired or invalidated
so long as the economic or legal substance of the transactions contemplated hereby is not affected in any manner materially adverse to any party. Upon 

  
 19 

 
such a determination, the parties shall negotiate in good faith to modify this Agreement so as to effect the original intent of the parties as closely as possible in an acceptable manner so that
the transactions contemplated hereby be consummated as originally contemplated to the fullest extent possible. 
 Section 4.11.
Confidentiality. Each Shareholder agrees that it will use, and will cause each of its affiliates, and each of its and their respective partners, members, managers, shareholders, directors, officers, employees and agents (collectively,
“Agents”) to use, all commercially reasonable efforts to maintain the confidentiality of all confidential information disclosed to it by the Company and will not, without the prior written consent of the Company, use such
confidential information other than in connection with the transactions contemplated herein. The foregoing shall not apply to confidential information that (a) is known or becomes known to the public in general (other than as a result of a
breach of this Section 4.11 by such Shareholder), (b) is or has been independently developed or conceived by the Shareholder without use of the Company’s confidential information, or (c) is or has been made known or disclosed to the
Shareholder by a third party without a breach of any obligation of confidentiality such third party may have to the Company; provided, however, that any Shareholder may disclose confidential information (i) to its attorneys,
accountants, consultants, and other professionals to the extent necessary to obtain their services in connection with monitoring its investment in the Company; (ii) to any prospective purchaser of any Registrable Securities from such
Shareholder, if such prospective purchaser is not a competitor to the Company (as determined in good faith by the Company’s Board of Directors) and agrees to be bound by the provisions of this Section 4.11; (iii) to a member, partner,
stockholder or wholly owned subsidiary of such Shareholder in the ordinary course of business, provided that such Shareholder informs such Person that such information is confidential and directs such Person to maintain the confidentiality of such
information; or (iv) as may otherwise be required by law. Without limiting the generality of the foregoing, in no event shall any Shareholder knowingly use any confidential information regarding the Company or its business acquired by such
Shareholder (directly or indirectly) in its capacity as a Shareholder which would result in a competitive disadvantage to the Company. Each Shareholder further agrees that any notice received pursuant to this Agreement, including any notice of a
proposed public offering, postponement of an offering or other similar notice regarding the Company’s securities, is confidential information and that any trading in securities of the Company following receipt of such information may only be
done in compliance with all applicable securities laws. 
 Section 4.12. Independent Nature of Shareholders’ Obligations and
Rights. The obligations of each Shareholder hereunder are several and not joint with the obligations of any other Shareholder hereunder, and no Shareholder shall be responsible in any way for the performance of the obligations of any other
Shareholder hereunder. Nothing contained herein or in any other agreement or document delivered at any closing, and no action taken by any Shareholder pursuant hereto or thereto, shall be deemed to constitute the Shareholders as a partnership, an
association, a joint venture or any other kind of entity, or create a presumption that the Shareholders are in any way acting in concert with respect to such obligations or the transactions contemplated by this Agreement. Each

  
 20 

 
Shareholder shall be entitled to protect and enforce its rights, including the rights arising out of this Agreement, and it shall not be necessary for any other Shareholder to be joined as an
additional party in any proceeding for such purpose. 
 [Signature pages follow.] 

  
 21 

 IN WITNESS WHEREOF, the parties hereto have duly executed this Agreement or have caused this
Agreement to be duly executed by their respective authorized officers as of the day and year first above written. 
  

			
	AURIS MEDICAL HOLDING AG
		
	By:	 	  

	Name:	 	
	Title:	 	
		
	By:	 	  

	Name:	 	
	Title:	 	

 [Signature page to the Registration Rights Agreement] 

  
 22 

			
	[—]
		
	By:	 	  

	Name:	 	
	Title:	 	
	
	Address for Notices:
	[Address]
	[Fax number]
	[Email address]
	
	With Copies of Notices to:
	
	[—]

 [Signature page to the Registration Rights Agreement] 

  
 23 

 Exhibit A 

Joinder to Registration Rights Agreement 

This Joinder Agreement (this “Joinder Agreement”) is made as of the date written below by the undersigned (the
“Joining Party”) in accordance with the Registration Rights Agreement dated as of [—] (as the same may be amended from time to time, the “Registration Rights
Agreement”), among Auris Medical Holding AG and the Shareholders party thereto. Capitalized terms used, but not defined, herein shall have the meaning ascribed to such terms in the Registration Rights Agreement. 

The Joining Party hereby acknowledges, agrees and confirms that, by its execution of this Joinder Agreement, the Joining Party shall be
deemed to be a party to the Registration Rights Agreement as of the date hereof as a “Permitted Transferee” of a Shareholder thereto, and shall have all of the rights and obligations of a “Shareholder” thereunder as
if it had executed the Registration Rights Agreement. The Joining Party hereby ratifies, as of the date hereof, and agrees to be bound by, all of the terms, provisions and conditions contained in the Registration Rights Agreement (including, without
limitation, Section 4.01 thereof). 
 IN WITNESS WHEREOF, the undersigned has executed this Joinder Agreement as of the date written
below. 
 Date: [—] 

 

			
	[NAME OF JOINING PARTY]
		
	By:	 	  

	Name:	 	
	Title:	 	
	
	Address for Notices:
	[Address]
	[Fax number]
	[Email address]

Source: [{"source": "alea-institute/alea-institute/kl3m-data-edgar-agreements/train-00233-of-00352.parquet"}, [{"source": "alea-institute/alea-institute/kl3m-data-edgar-agreements/train-00233-of-00352.parquet"}]]