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60 Possible Causes for Sensorineural Hearing Loss May Be Presenting Feature

  • Congenital Deafness

    Features may range from those with classical Pendred syndrome presenting with goitre and prelingual profound sensorineural hearing loss, to those with absence of goitre, normal[bmb.oxfordjournals.org] More recently, it has become apparent that the clinical presentation of individuals with mutations in this gene is highly variable.[bmb.oxfordjournals.org]

  • Susac Syndrome

    […] to retinal branch occlusions, and sensorineural hearing loss.[ncbi.nlm.nih.gov] hearing loss at low and medium frequencies 1-8 branch retinal artery occlusions leading to scotomata and vision distortion 1-8 All clinical features may not be present at[radiopaedia.org] All clinical features may not be present at the same time, and may in fact fluctuate over a 1-2 year period, which often leads to delay in diagnosis [ 5 ].[casereports.in]

  • Coronary Cataract

    Fechtner’s syndrome, which is also reported as a variant of AS, shares with the latter the triad of nephritis, sensorineural hearing loss, and eye abnormalities, and presents[entokey.com] […] the additional features of macrothrombocytopenia and polymorphonuclear inclusions (called Döhle-like bodies).[entokey.com] May-Hegglin’s anomaly, Sebastian’s syndrome, and Epstein’s syndrome are macrothrombocytopenias caused by distinct mutations of MYH9; however, unlike Fechtner’s syndrome, they[entokey.com]

  • Familial Spontaneous Pneumothorax

    Mild spondyloepiphyseal dysplasia may also be present, and the hearing loss may be conductive and sensorineural.[nature.com] The presence of some clinical features—including retinal detachment, cataract, sensorineural hearing loss, early-onset degenerative joint disease, and certain skeletal abnormalities—is[nature.com]

  • Otosclerosis

    hearing loss.[ncbi.nlm.nih.gov] Recognition of these MRI features is important as MRI may be the first modality of investigation, especially when patients present with symptoms indicative of sensorineural[ncbi.nlm.nih.gov] Increased signal on T(2) weighted images may also be seen.[ncbi.nlm.nih.gov]

  • Cryopyrin-Associated Periodic Syndrome

    Characteristically Muckle-Wells syndrome is associated with the early development of progressive sensorineural hearing loss.[dermnetnz.org] The clinical features of the acute attacks of Muckle-Wells syndrome may include: Fever – not present in all cases, but when present is usually higher than in FCAS (39-40C)[dermnetnz.org]

  • CINCA Syndrome

    The CNS manifestations include chronic aseptic meningitis leading to brain atrophy, mental delay and sensorineural hearing loss.[ncbi.nlm.nih.gov] These manifest themselves in three principal ways: chronic meningitis, involvement of both the optic tract and eye, and sensorineural hearing loss.[en.wikipedia.org] Chronic polyarthritis and alteration of the growth cartilage also may be present.[ncbi.nlm.nih.gov]

  • Autosomal Recessive Deafness 76

    Features may range from those with classical Pendred syndrome presenting with goitre and prelingual profound sensorineural hearing loss, to those with absence of goitre, normal[academic.oup.com] More recently, it has become apparent that the clinical presentation of individuals with mutations in this gene is highly variable.[academic.oup.com]

  • Autosomal Dominant Deafness 11

    Features may range from those with classical Pendred syndrome presenting with goitre and prelingual profound sensorineural hearing loss, to those with absence of goitre, normal[academic.oup.com] More recently, it has become apparent that the clinical presentation of individuals with mutations in this gene is highly variable.[academic.oup.com]

  • Autosomal Dominant Deafness 64

    Features may range from those with classical Pendred syndrome presenting with goitre and prelingual profound sensorineural hearing loss, to those with absence of goitre, normal[academic.oup.com] More recently, it has become apparent that the clinical presentation of individuals with mutations in this gene is highly variable.[academic.oup.com]

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