Sensorineural deafness

Complex V catalyzes the phosphorylation of ADP to ATP. Deficiencies of complex V are rare and clinical presentation is usually nonprogressive muscle weakness. One subject included growth retardation, sensorineural deafness, and involvement of basal ganglia, which are occasional features of various other respiratory chain abnormalities. 

Roman GC An epidemic in Cuba of optic neuropathy, sensorineural deafness, peripheral sensory neuropathy and dorsolateral myeloneuropathy. J Neurol Sci 127 11-28,1994. 

Dexamethasone given i.v. and early appears to reduce long-term neurological sequelae, especially sensorineural deafness, in infants and children. There is not, however, general agreement about the use of dexamethasone for meningitis in adults. 

Bassi MT, Ramesar RS, Caciotti B et al. X-linked late-onsct sensorineural deafness caused by a deletion involving OAl and a novel gene containing WD-40 repeats. Am J Hum Genet 1999 ... 

Serious adverse effects of aminobisphosphonates were reported in otospongiosis. Of two patients with stapedial otosclerosis and sensorineural hearing loss one developed total bilateral deafness and the other retained minimal auditory function in the low frequencies (25). It was recommended that sensorineural deafness in selected patients with otosclerosis should be treated with sodium fluoride, and that patients with Paget s disease treated with aminobisphosphonates should be closely monitored and the drug discontinued immediately if hearing deteriorates. Elsewhere, ototoxicity has been reported (SEDA-20, 440). 

The middle ear should be carefully protected against chlorhexidine solutions in preoperative skin disinfection in otolaryngology. Severe sensorineural deafness occurred in 14 of 97 patients who underwent myringoplasty (13). The only common factor in all these patients was the preoperative skin disinfection of the ear with 0.5% chlorhexidine in a 70% alcoholic solution. 

Bicknell PG. Sensorineural deafness following myringoplasty operations. J Laryngol Otol 1971 85(9) 957-61. 

Ocular hypertelorism. Pulmonic stenosis, Abnormahties of the genitalia. Retarded growth, and sensorineural Deafness (36). A CT scan showed diffuse interstitial injury characterized by thickening of the intralobular septa, with areas of ground-glass pattern. Flecainide was withdrawn and within 2 weeks the changes on CT scan had almost completely disappeared. 

Two reports of moderate to severe sensorineural deafness after metronidazole therapy, which resolved slowly after therapy ended suggest that this is an additional adverse effect (13). Both cases of deafness were preceded by tinnitus, which may be a warning to withdraw the drug. 

IV [al(IV)l2a2(IV) other forms are also known Basement membrane Alport Syndrome characterized by nephritis and sensorineural deafness... 

Pendreds syndrome is an autosomal recessive disorder characterized by sensorineural deafness, goiter, and impaired iodide organification. 

Hypothyroidism can result in depression, pseudodementia and, in severe cases, myxedema coma. Conversely, it can also lead to inappropriate euphoria, depression and frank psychosis (Lichtenstein, 1980). Other higher center problems include sensorineural deafness, and the mechanism is thought to be due to the deposition of gly-cosamino glycan and bone hypertrophy, but this can be difficult to confirm. Severe hypothyroidism can result in severe mental retardation (cretinism) in children. Grandmal seizure is not uncommon. It is thought to be due to the failure to excrete water, resulting in severe hyponatremia and occurs in about 25% of cases (Myers and Hays, 1975). 

C. Tinnitus and irreversible sensorineural deafness occurs between 4 and 16 hours after ingestion in adults, but deafness may be delayed for several days in children. 

Sensory systems Hearing Hearing loss can occur after the use of opioids. In a retrospective case series review, bilateral rapidly progressive profound sensorineural deafness and macrocytosis were reported in association with codeine in 10 patients [37 ]. All had a history of long-term codeine use and abuse. The authors proposed that the presence of macrocytosis in all the cases suggested a vascular pathology. 

Freeman SRM, Bray ME, Amos CS, Gibson WPR. The association of codeine, macrocytosis and bilateral sudden or rapidly progressive profound sensorineural deafness. Acta Otolaryngol 2009 129(10) 1061-6. 

Evidence of purine overproduction in childhood has led to detection of superactive PRPP synthetases in 2 families which are of special interest for several reasons, First, the hemizygous affected males in these families show severe sensorineural deafness in addition to uric acid overproduction. Second, the mothers of these boys share both the metabolic and hearing abnormalities with their sons, and one of these women has had both acute gouty arthritis and uric acid urolithiasis. Finally, as discussed below, the functional derangement in the enzyme of one of the families is unusually marked with more severe metabolic consequences of PRPP synthetase superactivity which might explain the childhood clinical onset, the development of gout in the mother, and even the associated deafness. 

Cochlear Implants. Cochlear implants (Cl), by fer the most successful sensory neural prostheses to date, have penetrated the mainstream therapeutic arsenal. Their popularity is rivaled only by the cardiac pacemakers and deep brain stimulation (DBS) systems. Implanted in patients with sensorineural deafness, these devices process sounds electronically and transmit stimuli to the cochlea. A Cl includes several components a microphone, a small speech processor that transforms sounds into a signal suitable for auditory neurons, a transmitter to relay the signal to the cochlea, a receiver that picks up the transmitted signal, and an electrode array implanted in the cochlea. Individual results vary, but achieving a high degree of accuracy in speech perception is possible, as is the development of language skills. 

Large variety of sub-strains presenting with varying degrees of age-related sensorineural deafness occurring early in life (Erway et al. 1993 Johnson et al. 2000 Ohlemiller 2006). 

An infantile profile, essentially involving a. retinopathy often leading to early blindness, b. sensorineural deafness, c. hepatic dysfunction that may involve symptoms of portal hypertension, d. mental deficiency, e. (often) failure to thrive, f. dysmorphic signs. 

Seminiferous tubule dysgenesis 567 Sensorineural deafness 197, 494 Sensory disturbances 497 Sepsis 146, 340 Sex/sexual... 

---