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12 Possible Causes for Gradual Progression of Hearing Loss, Sensorineural Hearing Loss May Be Presenting Feature

  • 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] loss Generally begins in 3rd decade Hearing loss is generally unilateral and gradual MRI to diagnose Other tumors are associated Molecular testing available X-linked Alport[en.wikibooks.org] Most affected individuals noticed hearing loss in their first decade of life after complete speech acquisition, with subsequent gradual progression.[academic.oup.com]

  • Otosclerosis

    hearing loss.[ncbi.nlm.nih.gov] This far-advanced otosclerosis often presents with a family history of hearing loss or a gradually progressive hearing loss that starts early in adult life.[emedicine.medscape.com] A history of gradually progressive hearing loss in one ear, beginning in a patient's late teens or early adulthood, suggests otosclerosis.[marshfieldclinic.org]

  • Susac Syndrome

    […] to retinal branch occlusions, and sensorineural hearing loss.[ncbi.nlm.nih.gov] Hearing loss was fluctuant but gradually progressive over nine months, to bilateral profound 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]

  • CINCA Syndrome

    The CNS manifestations include chronic aseptic meningitis leading to brain atrophy, mental delay and sensorineural hearing loss.[ncbi.nlm.nih.gov] progressive loss of hearing Symptoms increase when exposed to cold temperatures, stress; Frequently continuous symptoms NOMID/CINCA (severest manifestation) Daily rashes[autoinflammation-reference-center-charite.de] […] arthritis Progressive (increasing) hearing loss Eye infection (uveitt) Neonatal Onset Multisystem Inflammatory Disorder (NOMID) CINCA Syndrome (Chronic Infantile Neutrophilic[bindevevssykdommer.no]

  • 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] Worsening speech or school performance may herald long-standing or progressive mild-to-moderate hearing loss.[emedicine.medscape.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] The hearing loss initially involved high frequencies, with subsequent gradual progression to a severe level involving all frequencies.[link.springer.com] More recently, it has become apparent that the clinical presentation of individuals with mutations in this gene is highly variable.[academic.oup.com]

  • Maternally-Inherited Diabetes and Deafness

    Conclusions: MIDD syndrome should be considered in patients presenting with a clinical syndrome of sensorineural hearing loss and diabetes mellitus.[neurology.org] Hearing gradually deteriorated; the progression rate ranged from 1.5 to 7.9 dB per year.[ncbi.nlm.nih.gov] His mother also had hearing impairment that gradually progressed with age. DNA samples were not obtained from other family members.[nature.com]

  • Camurati-Engelmann Syndrome

    Mild spondyloepiphyseal dysplasia may also be present, and the hearing loss may be conductive and sensorineural.[nature.com] Some patients may note abrupt unilateral hearing loss, facial nerve paralysis, or vision loss; others may experience gradual or fluctuating unilateral or bilateral symptoms[jamanetwork.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]

  • Vestibular Aqueduct Dilated

    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] One is a gradual progressive stepwise hearing loss, but with fluctuations.[hearinglosshelp.com] They tend to be fearful of implanting their ears because they have lived their whole life with gradual or sudden progressions of hearing loss, and they use up all of that[audiologyonline.com]

  • Conductive Deafness - Malformed External Ear Syndrome

    All types have progressive vision loss. Waardenburg Hearing loss may be present in one or both ears and is a sensorineural type of loss.[rchsd.org] A progressive nerve disease which causes gradual decrease of muscle function in legs. The hearing loss is sensorineural and usually is noted in adolescence ...[ibis-birthdefects.org] Pendred Hearing loss may be mixed or sensorineural and may be progressive. Abnormal inner ear anatomy and thyroid enlargement can also be present.[rchsd.org]

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