Hearing Preservation During Cochlear Implantation and Electroacoustic Stimulation in Patients With SLC26A4 Mutations.

BACKGROUND AND OBJECTIVES: Patients with SCL26A4 mutations presenting with Mondini deformity and enlarged vestibular aqueduct (EVA) tend to have comparable residual hearing. Although cochlear implantation (CI) produces good results in this group, deterioration of residual hearing can be an adverse event after surgery due to accompanying cochlear malformation and perilymph leakage during cochleostomy. The purpose of this study was to investigate if CI in patients with SCL26A4 mutations via the round window (RW) approach could achieve preservation of residual hearing, and to evaluate their speech reception with electroacoustic stimulation (EAS). SUBJECTS AND METHODS: This is a retrospective chart review of eight patients with bilateral EVA, who were bi-allelic patients with SCL26A4 mutations. CI was performed in all patients by a single surgeon using the RW approach. Audiological results were compared before and after implantation. RESULTS: Additional hearing loss after CI was less than 10 dBHL in five out of eight patients. Average hearing deterioration after CI was 8.75 dB (range, 0-26). Six out of eight patients used EAS mode after CI. The acoustic stimulation frequency ranged from 271 to 438 Hz. Patients showed better speech recognition in quiet and in noise using EAS mode compared with electrical stimulation alone. CONCLUSIONS: Preservation of residual hearing could be achieved after CI in patients with the SLC26A4 mutation via the RW approach. For successful preservation of residual hearing, application of newly-developed soft electrode and meticulous surgical is necessary. Our study showed that patients with the SLC26A4 mutation can be good candidates for EAS surgery.

Ventilation tube insertion is not effective to the treatment of hearing impairment in pediatric patients with Cornelia de Lange syndrome.

OBJECTIVE: Cornelia de Lange syndrome (CdLS) is a multiple developmental disorder including hearing loss. The hearing impairment in CdLS patients is not only sensorineural but also conductive hearing loss (CHL). The aim of this study was to elucidate hearing loss causes in CdLS patients and evaluate the effect of ventilation tube (v-tube) insertion in the cases of CHL. METHODS: Thirty-two patients clinically diagnosed with CdLS were enrolled and analyzed with retrospective case review. Audiologic evaluations and imaging studies such as a temporal bone computed tomogram or brain magnetic resonance imaging (MRI) were performed for all patients. Hearing rehabilitation such as ventilation tube insertion, hearing aid fitting, or cochlear implantation was chosen depending on the audiological condition. RESULTS: Among 32 CdLS patients who underwent auditory brainstem response test, 81.2% presented hearing loss. Imaging studies showed that only middle ear lesions without inner ear anomalies were identified in 56.3%. Notably, the soft tissue lesion in middle ear was identified even in the neonatal MRI. When 7 patients were thought to have CHL due to otitis media with effusion, v-tube insertion was applied first. However, v-tube insertion rarely improved CHL postoperatively. Moreover, middle ear lesion was not fluid effusion but soft tissue lesion according to the intraoperative finding. These lesions were not eradicated even after revision surgery of v-tube insertion. CONCLUSION: V-tube insertion is not effective to improve hearing or eradicate otitis media with effusion in CdLS patients.

Intraindividual comparison of psychophysical parameters between perimodiolar and lateral-type electrode arrays in patients with bilateral cochlear implants.

OBJECTIVE: Perimodiolar electrode arrays were developed to improve stimulation of specific neuronal populations and to decrease power consumption; however, they can damage the cochlear structure. We examined and compared psychophysical parameters of perimodiolar and lateral-type electrode arrays in patients who received a different type of bilateral cochlear implant (CI) in each ear. STUDY DESIGN: Retrospective analysis. SETTING: Tertiary referral center. PATIENTS: Eight child patients (three males, five females) received a different CI in each ear (perimodiolar array and lateral array). They received the CIs sequentially (n = 7) or simultaneously (n = 1). INTERVENTIONS: Diagnostic, therapeutic, and rehabilitative. MAIN OUTCOME MEASURES: Electrically evoked compound action potential, threshold level, comfort level, and dynamic range (DR) of the basal, mid, and apical electrodes were compared. We also surveyed battery consumption for each device. RESULTS: Electrically evoked compound action potential threshold, threshold level, and comfort level were lower for the perimodiolar-type electrode array than for the lateral-type electrode array in most patients. However, the DR for the perimodiolar array was narrower than for the lateral array. For most patients, there was little difference in battery life. CONCLUSION: Although the level of electrical energy required for auditory stimulation seems to be lower for the perimodiolar electrode array than for the laterally placed array, the DR was wider and the amount of battery consumption was similar. The electrode array should be chosen by considering various patient factors, such as residual hearing.

Tinnitus related to eyelid blinking.

Tinnitus can be generated by various causes, including vascular or myogenic factors and sensorineural auditory structures. Tinnitus due to repetitive contraction of the paraauditory muscles is a rare condition. We present a case of bilateral tinnitus concomitant with eyelid blinking. Otoscopy revealed normal tympanic membranes; however, inward movement of the tympanic membrane was visible whenever the patient blinked. Long-time-based tympanometry measuring static compliance revealed a cogwheel or saw-toothed pattern associated with movement of the tympanic membrane related to eyelid blinking. The patient was managed with pharmacological treatment combined with assurance and was relatively well controlled.

Severe to profound hearing loss in patients with progressed Alport's syndrome.

CONCLUSION: The concept of hearing loss severity must be redefined, as there is a clear need for more active hearing management in Alport's syndrome patients with severe and profound hearing loss. OBJECTIVES: Sensorineural hearing loss (SNHL) caused by Alport's syndrome generally does not exceed 60-70 dB, because a cochlear lesion is responsible for this hearing loss. Careful management of renal function improves the prognosis and the longevity of Alport's syndrome patients; it is useful to reassess SNHL caused by Alport's syndrome. PATIENTS AND METHODS: Thirty-two patients with Alport's syndrome were analyzed retrospectively. Pure tone audiograms (PTAs), speech audiograms, and transiently evoked otoacoustic emissions (TEOAEs) were performed. Hearing loss severity was compared to duration of disease and severity of renal dysfunction. We also evaluated the correlation between OAEs and PTAs according to the hypothesis that evoked OAEs would be abnormal even in early stage SNHL in Alport's syndrome. RESULTS: The level of hearing was positively correlated with disease duration. The hearing of the end-stage renal disease (ESRD) group, whose hearing threshold could exceed 70 dB, was worse than that of the non-ESRD group. OAEs were found in patients with normal hearing and mild hearing loss and had no significant early detection value.