Information has been received from a physician concerning a male pt who was vaccinated with a dose of MMRII. Subsequently, the pt experienced loss of hearing. On 12/27/01, the physician reported that the pt was was now 2.5 years old and he was going to be seen for his loss of hearing. The physician was unsure when the pt received his vaccination. Follow-up information from the physician, who is an ear specialist, reported that he saw the pt and has confirmed his hearing loss. The physician has also determined that the pt has some neurological issues and he referred the pt to a neurologist to determine if the neurological issues may have a role in his hearing loss. The physician reported that he does not know if the child's hearing loss is associated with the MMRII, however the pt's mother is convinced that the hearing loss was due to the vaccine. Upon internal review, the pt's loss of hearing and neurological issues were considered to be an "Other Important Medical Event". Additional information has been requested. Follow up info indicated that on 1/29/02, the pt was referred for a neurologic evaluation in the context of his acute progressive hearing loss. At this time, the pt was a 34 month old toddler whose mother noted that as a newborn he had normal hearing, then in September 2001, four momths ago, he was tested and had normal hearing. In December 2001, he was diagnosed wiht a 100% right-sided hearing loss and a 60% left-sided hearing loss. He was recently fitted with hearing aids. About a month ago in December 2001, he had a CT scan done to see if he had an anatomical abnormality to account for his hearing loss. His mother also reported that he had multiple words at one year of age and then had progressive loss of vocabulary between the ages of 15 ad 18 months of age. At this point he was unable to make any choices in his words. His mother believes that he does know some colors and for instance can match colors, but it is really difficult ot assess his knowledge base because of his hearing loss. With the regards to the other areas of developemtn, the pt walked at around 11 months of age. His mother feels that he is quite coordinated. He is energetic, has good memory level and is able to have good balance and coordination, has good fine motor skills, with no tremor. He has had no difficulty in terms of his motor skills and no loss of these skills. His mother notes that in August he began having difficulty with some abnormaltiies of mouth movements. He was noted to start drooling, his tongue protruded on his lower lip and he had oral motor problems. She now notes that at his tongue and lower lip will seem to have quivering ro tremulousness. His parents feel that this was getting worse. He has not experienced difficulty with chewing or swallowing, he has no change in his voice quality, does not have difficulty with sleeping, but parents are concerned about this new change. the pt's history was reported and it was ntoed that the pt was born to a 28 year old gravida II mother at term. Her pregnancy was complicated by several urinary tract infections. She was afebrile at the time and treated with antibiotics. His mother had varicella when she was 7 months pregnant. He was born at term via vaginal delivery with a birth weight of 7 ob. 15 oz. His Apgars were 9 and 10. There were no perinatal difficulties Subsequently the pt was quite healthy, except for one episode of RSV in his first year of life. He was hospitalized for pneumonia in January 2001 and treated with oxygen and antibiotics. There has ben not recent significat infection noted. His father is currently healthy, although when he was a teenager he had surgery for an orbital fibrous dysplasia, which was compressing his optic nerve and causing him severe headache pain. His father was adopted and his father history is not otherwise known. His mother has been healthy. There are no neurological problems noted in her family. The pt has a 4 year old sister who is in good health and developing normally. Upon review of his body systems, his vision was reportedly normal and he has no cardiac difficulties. He has not had bowel or bladder problems and no recurrent constipation. His muscle strength has been good. He has had no recurrent skin rashes and no known significant birthmarks. He has no allergies that are known and he is not taking any daily medications. He is a cuddly toddler, seeks his parents for comfort and has not had frequent temper tantrums and his parents feel that his behavior has been "normal." He has been responding to his therapies, although htey had noted some degree of frustration. A physical examination was performed and his weight is 49 pounds, height is 39.5 inches and his head circumference is 51.5cm which is at the 75th centile for his age. He does have some difficulties cooperating with the examination today. He has clear nasal rhinorrhea. His voice quality is strong, with no hoarseness noted. His chest is clear. He has no cardiac murmur. His abdomen is soft with no hepatomegaly noted. He idid not have any axillary or groin hyperpigmentation. He is a normal circumcised male with genetelia appearing to be normal in size. There are no dysmorphic features that are seen. His neurological examination showed that his extraocular movements are full. His optic disks were not well visualized. There was no afferent pupillary defect seen. Facies are symmetric as he smiles or cries and he appears to have normal strength, being able to bury his eyelashes bilaterally. he did not have excessive drooling noted. There were times when his tonue would rest on his lower lip and the physician did not see fasciculations or true chin quivering as noted by his parents. he did attempt to say several words, which the physician was able to understand. his palate elevates symmetrically. Motor maneuver, does have some mild pronation and intoeing as he walks, but certain ataxia was not seen. He uses both hadns to select objects individually well, raises his hands nicely above his head and executes good strength in his arms and legs. Reflexes are one to two and symmetric. Plantar responses withdrawel bilaterally. The mom was examined and does not demonstrate hyperreflexia or extensor plantar responses. His father's skin was examined as he has one irregular cafe au lait spot over his right abdomen. The physician's impression was that "the pt has new onset of subacute progressive hearing loss, which is bialteral. Certainly there are a number of syndromes which could go along with this diagnosis. If his father's history is significant, then an osteoopatrosis or metaphyseal dyplasia syndrome might be considered. It would be much less likely to have a phacomatosis, such as neurofibromatosis type II, accounting for this disorder. In the differential diagnosis, aside from an anatomic abnormality, we would also consider a white matter disturbance, such as leukodystrophy. His mother evidently has two brothers who are in their late 20's and are normal. Her examination does not indicate hyperreflexia or any abnormalities that would go along with adrenal leukodystrophy, but we will investigate this further. A post infectious demyelinating disorder could also be ocnsidered although historically there is not preceding event for the pt. Other metabolic disorders, such as Refsum's disease, or abnormality of phytanic acid metabolism should be looked at. Certainly there are multiple genetic disorders, such as Wardenburg syndrome adn many other disorders involving the kidneys and otpic nerves which we will also consider down the line. The physician felt that a cranial MRI should be performed to look at the white matter and make certian that there are no structural abnormalities. If this is normal, then the physician would recommend that we go forward and have a urinalysisi done to look for sugar or protein, as well as phytamic acid. In addition, the physician would recommend a dialated opthalmologic examination to look for retinitis pigmentosa and to measure the size of the optic nerve to make certain that there wa sno optic atrophy involvement." On 2/5/02 the pt had a MRI with and without contrast because of his progressive hearing loss. He was given "Nebutol" 1.62 mg intravenously for the procedure. His pulse oximetry was monitored and it never dropped below 94%. The conclusion was a normal MR scan. Upon internal review, the pt's loss of hearing and neurological issues were considered to be other important medical events (OMIC). Additional info has been requested. Follow up information received from a physician indicated that she had not seen the patient again for follow up and that she had seen him only once. Follow up information received from the ear specialist indicated that the patient was under the care of a pediatric neurologist for the tongue fasciculations. He indicated that they have no definitive neurological diagnosis at this point. It was noted again that the recent MRI scan was normal. The physician reported that the patient suffered from what appeared to be a sensorineural hearing loss, which he did not expect to improve over time. As far as he could tel, there had been no progression and the patient was using his hearing aids with good improvement, and if, in fact, it was related to the immunization, would not expect progression either. He referred to the situation as difficult, and reported once again that the only association with the MMR (second generation) and hearing loss was the mother's temporal association. Additional information is not expected.