Sensorineural hearing loss in Bartter syndrome

Abstract

ABSTRACTBackground: Bartter syndrome is a rare inherited case characterized by autosomal recessive and has few different types. Diagnosis is established by laboratory findings, namely hypokalemic metabolic alkalosis, and normotensive. Hearing loss indicates Bartter syndrome type IV. Purpose: To report a case of Bartter syndrome with delayed speech. Case report: A seven years old girl with delayed speech and recurrent hypokalemia was referred to the Otolaryngology Head and Neck Surgery Department, Dr. Cipto Mangunkusumo Hospital, in order to evaluate the hearing level and treatment needed. Based on the Otoacoustic Emission (OAE), Brainstem Evoked Response Audiometry (BERA), and Auditory State Steady Response (ASSR), the diagnosis was profound bilateral sensorineural hearing loss and proceeded with hearing aid and also speech occupational therapy. Clinical question: Is there a relationship between Bartter’s syndrome and the incidence of hearing loss? Review method: Literature review through PubMed, Cochrane, and EBSCO, using keywords such as the impacts of Bartter syndrome on hearing loss, and sensorineural hearing loss in Bartter syndrome case. Result: Following screening of double publication and based on clinical questions over the past five years, only one relevant literature was found. Conclusion: Audiological assessment should be done in all Bartter syndrome’s cases. Early intervention and timely audiological rehabilitation could improve the quality of life in of such children.