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Info


A. Infectionnavigator

  1. Syphilis
  2. Bacterial Meningitis - especially in children
  3. Rubella
  4. Cytomegalovirus (CMV)

B. Drug Associatednavigator

  1. Aminoglycosides
  2. Loop Diuretics - especially ethacrynic acid
  3. Cisplatin
  4. Salicylates

C. Traumanavigator

  1. Skull Fracture - temporal bone through inner ear
  2. Noise Exposure
  3. Radiation
  4. Perilymphatic fistulas

D. Circulatorynavigator

  1. Vertebrobasilar Insufficiency
  2. Thromboembolic Disease
  3. Hypercoagulable State
  4. Hypercholesterolemia

E. Tumors of the Cerebellopontine Angle [2] navigator

  1. Acoustic Neuroma (>90% of these tumors; also called vestibular schwannoma)
  2. Arachnoid Cyst
  3. Cavernous Hemangioma
  4. Choiroid Plexus Papilloma
  5. Epidermoid
  6. Epithelial Cyst
  7. Facial Nerve Schwannoma
  8. Lipoma
  9. Meningioma
  10. Metastatic Tumors
  11. Teratoma

F. Rheumatologic Disease navigator

  1. Giant-Cell Arteritis (GCA)
  2. Systemic Lupus Erythematosus (SLE)
  3. Polyarterteritis Nodosa (PAN)

H. Genetic [2]navigator

  1. Congenital deafness occurs in ~0.1% of live births
  2. About 50% of congenital cases are hereditary
  3. A number of syndromes have been characterized and account for ~20% of hereditary cases
  4. Non-Syndromic Recessive Deafness [6]
    1. Acounts for ~80% of hereditary deafness
    2. Over 20 genes which, when mutated, can cause deafness have been discovered
    3. Mutations in the gene GJB2 encoding gap junction protein connexin 26 have been found among Ashkenazi Jews with nonsyndromic recessive deafness
    4. Mutations in gene GJB6 encoding connexin 30 is second most common cause of non- syndromic prelingual hearing loss in Spanish patients [9]
  5. Alezzandrini's Syndrome (unilateral deafness and retinitis, facial vitiligo)
  6. Alport's Syndrome (renal abnormalities and SNHL) [10]
  7. Bernard-Soulier Syndrome (similar to Epstein Syndrome)
  8. Epstein's Syndrome (nephritis, deafness, and large platelets with thrombocytopenia) [3]
  9. Jervell and Lange-Nielsen Syndrome [4]
  10. Pendred's Syndrome (hypothyroidism and SNHL)
  11. Usher's Syndrome (Retinitis pigmentosa blindness and SNHL)
  12. Waardenburg's Syndrome
  13. Maternal Inherited Diabetes with Deafness (MIDD) [7]
    1. Due to point mutation in miochondrial DNA at position 3243
    2. Leads to mutant transfer RNA leucine
    3. Mainly due to defect in secretion of insulin, rather than insulin insensitivity
    4. Maternal family history of DM found in 73% of probands
    5. Average age of DM onset ~40 years
    6. Most patients progressed to insulin dependency within 10 years
    7. Neurosensory hearing loss present in nearly all patients
    8. Muscle dysfunction common: 43% myopathy, 15% cardiomyopathy
    9. Macular pattern dystrophy found in most patients
    10. Prevalance of kidney disease 28%
  14. Muckle-Wells Syndrome [8]
    1. Related to familial cold urticaria but not triggered by cold
    2. Rare autosomal dominant disorder due to mutations in CIAS1 gene on chr 1q44
    3. CIAS1 is a pyrin domain protein with likely role in inflammation
    4. Symptoms develop by age 1 year
    5. Symptoms include nonpruritic nonurticarial maculopapular rash
    6. Rash may be accompanied by chills, fever, arthralgias, conjunctivitis
    7. Myalgias, headache and fatigue can occur
    8. progressive sensorineural deafness

G. Idiopathicnavigator

  1. Presbycusis
  2. Meiere's Disease
  3. Sudden idiopathic

H. Conductive Hearing Lossnavigator

  1. Cerumen Impaction
  2. Foreign Body
  3. Otitis externa
  4. Otitis media
  5. Cholesteatoma
  6. Otosclerosos
  7. Trauma with tympanic membrane perforation or hemorrhage
  8. Middle ear masses

I. Hearing Aids [5]navigator

  1. Analog Hearing Aids
  2. Crystal Clear Hearing Aid
  3. Programmable Hearing Aid
  4. Digital Hearing Aid

References navigator

  1. Nadol JB. 1993. NEJM. 329:1092 abstract
  2. Willems PJ. 2000. NEJM. 342(15):1101 abstract
  3. Cahill MR and Newland AC. 1997. Lancet. 349:1066 abstract
  4. Splawski I, Timothy KW, Vincent GM, et al. 1997. NEJM. 336(22):1562 abstract
  5. Hearing Aids. 1998. Med Let. 40(1028):62 abstract
  6. Morell RJ, Kim HJ, Hood LJ, et al. 1998. NEJM. 339(21):1501
  7. Guillausseau PJ, Massin P, Dubois-LaForgue D, et al. 2001. Ann Intern Med. 134(9):721 abstract
  8. Drenth JPH and van der Meer JWM. 2001. NEJM. 345(24):1748 abstract
  9. Del Castillo I, Villamar M, Moreno-Pelayo MA, et al. 2002. NEJM. 346(4):243 abstract
  10. Hudson BG, Tryggvason K, Sundaramoorthy M, Neilson EG. 2003. NEJM. 348(25):2543 abstract