The Outcome of Cochlear Implantation for Mitochondrial Disease Patients With Syndromic Hearing Loss
نویسندگان
چکیده
منابع مشابه
Mitochondrial syndromic sensorineural hearing loss.
Mitochondrial diseases (MD) are a clinically heterogeneous group of disorders that arise as a result of dysfunction of the mitochondrial respiratory chain. Sensorineural hearing loss (SNHL) is often associated to mitochondrial dysfunctions both in syndromic, nonsyndromic forms. SNHL has been described in association to different mitochondrial multisystemic syndromes, often characterized by an i...
متن کاملCochlear implantation for progressive hearing loss.
The concept of neural plasticity and the early natural abilities of hearing children to acquire speech and language without instruction have led many authorities to advocate cochlear implantation before the age of 5 years in congenital deafness. Older children therefore become lower priority for scarce public funds because they are perceived to have passed the "window of opportunity" to learn s...
متن کاملCochlear conductive hearing loss in patients with Meniere's disease.
OBJECTIVES Physiological studies with experimental models of endolymphatic hydrops in Meniere's disease demonstrate some pressure changes at the level of round and oval window. Interestingly, conductive type hearing loss was observed in some patients during Meniere episodes. A close auditory follow-up of these patients may provide a better understanding of hydrodynamic changes of the hydrops an...
متن کاملSyndromic Hearing Loss in Association with PTPN11-Related Disorder: The Experience of Cochlear Implantation in a Child with LEOPARD Syndrome
Hearing loss (HL) is one of the most frequent clinical manifestations of patients who suffer with multi-systemic genetic disorders. HL in association with other physical stigmata is referred to as a syndromic form of HL. LEOPARD syndrome (LS) is one of the disorders with syndromic HL and it is caused by a mutation in the PTPN11 or RAF1 gene. In general, 5 year old children who undergo cochlear ...
متن کاملCochlear implantation in autistic children with profound sensorineural hearing loss.
INTRODUCTION Cochlear implants have become the method of choice for the treatment of severe-to-profound hearing loss in both children and adults. Its benefits are well documented in the pediatric and adult population. Also deaf children with additional needs, including autism, have been covered by this treatment. OBJECTIVE The aim of this study was to assess the benefits from cochlear implant...
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ژورنال
عنوان ژورنال: Otology & Neurotology
سال: 2015
ISSN: 1531-7129
DOI: 10.1097/mao.0000000000000817