Age: 10.0
Sex: F
Vaccine: PPV
Symptoms: Acoustic stimulation tests abnormal, Cochlea implant, Condition aggravated, Deafness, Inner ear disorder
Clinical narrative: It was reported in a published article, five cases of postmeningitic deafness secondary to pneumococcal disease despite having received appropriate pneumococcal vaccinations. The 10 year old female patient was asplenic, a known risk factor for invasive pneumococcal disease. She had experienced pneumococcal meningitis 6 years earlier but recovered without complication. Preventive measures for meningitis included vaccination with a dose of PNEUMOVAX 23 (manufacturer unknown), appropriate to the patient's age and medical condition. The patient's meningitis reoccurred despite vaccination with PNEUMOVAX 23 five years later and daily oral penicillin. Profound deafness was diagnosed by using standard behavioral audiologic test methods on the fourth day of hospitalization. A three-dimensional SSFP MRI test that was performed six days after meningitis showed dramatically diminished T2 signal intensity within the turns of the cochlea, bilaterally. Surgical findings after twelve days of meningitis in the scala tympani of these ears revealed significant soft tissue; right ear: soft tissue filling basal turn, left ear: 4 mm of soft tissue. The patient underwent cochlear implementation (CI), bilaterally, between 01-DEC-2005 and 30-NOV-2007. The physicians relied on ABR/ASSR and imaging results alone to determine CI candidacy; the use of these techniques enabled the patient to undergo expedited evaluation for CI candidacy. An aggressive approach was used to optimize the likelihood of full electrode insertion in both ears, since the patient belonged to a population known to be at risk for progressive ossification. Pertinent medical history including pneumococcal vaccination status, results of auditory brainstem response (ABR)/ASSR testing and 3D SSFP MRI, degree of scala tympani obstruction, and achievement of electrode insertion constituted the mail focus of review. Bilateral cochlear implantation is of special importance in children with postmeningitic deafness. Despite complete electrode insertion, the patient was using only one device. The patient indicated that use of the processor in one of their ears was not useful despite multiple reprogramming sessions. Significant damage to the cochlear nerve by meningitis was suspected to be the underlying cause of these apparent ear differences. The preferred ear was not predictable preoperatively on the basis of imaging, audiologic testing, or intraoperative findings. Had only one ear undergone implantation and the second ear been addressed later, the obstruction found at the later surgery might have been more difficult to address. Conclusions: Pneumococcal vaccination had reduced but not eliminated childhood deafness secondary to pneumococcal disease. Auditory steady-state response testing and 3-dimensional steady-state free precession imaging are modalities that expedite candidacy evaluation of this population. Early bilateral simultaneous implantation increases the likelihood of binaural hearing and ensures implantation of the better ear in this population of children whose course is often complicated by formation of scar tissue and ossification within the cochlea. Additional information has been requested.