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. 2016 Apr;36(2):119-26.
doi: 10.14639/0392-100X-587. Epub 2016 Apr 29.

Idiopathic sensorineural hearing loss in the only hearing ear

S Berrettini  1   2 , A De Vito  1 , L Bruschini  1 , S Fortunato  1 , F Forli  1
Affiliations

Idiopathic sensorineural hearing loss in the only hearing ear

S Berrettini et al. Acta Otorhinolaryngol Ital. 2016 Apr.

Abstract
in English, Italian

A retrospective chart review was used for 31 patients with sudden, progressive or fluctuating sensorineural hearing loss (SHL) in the only hearing ear who had been consecutively evaluated at the ENT, Audiology and Phoniatrics Unit of the University of Pisa. The group of patients was evaluated with a complete history review, clinical evaluation, imaging exam (MRI, CT), audiologic tests (tone and speech audiometry, tympanometry, study of stapedial reflexes, ABR and otoacoustic emission) evaluation. In order to exclude genetic causes, patients were screened for CX 26 and CX30 mutations and for mitochondrial DNA mutation A1555G. Patients with sudden or rapidly progressive SHL in the only hearing ear were treated with osmotic diuretics and corticosteroids. In patients who did not respond to intravenous therapy we performed intratympanic injections of corticosteroid. Hearing aids were fitted when indicated and patients who developed severe to profound SHL were scheduled for cochlear implant surgery. The aim of this study is to report and discuss the epidemiology, aetiopathogenesis, therapy and clinical characteristic of patients affected by SHL in the only hearing hear and to discuss the issues related to the cochlear implant procedure in some of these patients, with regard to indications, choice of the ear to implant and results.

Uno studio retrospettivo è stato condotto su 31 pazienti, giunti all’osservazione della cinica ORL Audiologia e Foniatria dell’Università di Pisa dal 2007 al 2013, affetti da ipoacusia neurosensoriale improvvisa, fluttuante o progressiva nell’unico orecchio udente. L’intero gruppo di pazienti è stato valutato con una adeguata anamnesi, otomicroscopia, esami di imaging (TC RMN), test audiologici (audiometria tonale e vocale, impedenziometria, potenziali evocati uditivi e otoemissioni acustiche). Questo gruppo di pazienti è stato sottoposto anche a una valutazione genetica (ricerca mutazioni CX 26, CX 30 e DNA mitocondriale A1555G) e a test di laboratorio. I pazienti con ipoacusia improvvisa o rapidamente progressiva sono stati trattati con diuretici osmotici e corticosteroidi (endovena o intratimpanici). I pazienti che non hanno recuperato in maniera adeguata dopo il trattamento sono stati avviati a un percorso di protesizzazione. Nei pazienti con insufficiente resa protesica sono stati sottoposti ad impianto cocleare. Lo scopo di questo studio è quello di discutere l’epidemiologia, l’eziopatogenesi e le caratteristiche cliniche di pazienti affetti da ipoacusia neurosensoriale nell’unico orecchio udente e inoltre discutere le indicazioni e la scelta dell’orecchio da impiantare in questa categoria di pazienti.

Keywords: Cochlear implant; Only hearing ear; Progressive sensorineural hearing loss; Sensorineural hearing loss.

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