به نام خداوند جان و خرد

Back ground: METABOLIC BONE DISEASE IN OTIC CAPSULE

1-GENETICALLY MEDIATED AUTOSOMAL DOMINANT 2-VIRUSES: MEASLES SYMOTOMS: CHL – SNHL – MHL AGE: 10 – 45 years RACE – 10 % WHITE POPULATIONS 0.5% ASIANS 0.1% AFRICAN 75 – 80% BILATERAL

1- EARLY OTOSPONGIOTIC PHASE 2- LATER OTOSCLEROTIC PHASE

FREQUECY: HISTOLOGICALLY OTOSCLEROSIS: 10% CLINICALLY OTOSCLEROSIS: 1% MORTALITY & MORBIDITY: 1- CHL 2- SNHL 3- MHL 4-TINNITUS 5- VERTIGO

RACE: MORE COMMON IN WHITE PERSONS SEX: FEMALE -2 MALE -1 AGE: CLINICAL OTOSC. CAN MANFEST AS AGE 7 -8 YEARS MOST COMMONLY IN AGE 15 – 35 YEARS PICK INCIDECE: 3TH DECADE

1- HEARING LOSS: CHL – SNHL – MHL 2- TINNITUS 3- DIZZINESS 4- ROTATORY VERTIGO

1- PUR TONE AUDIOMETRY 2- TYMPANOMETRY 3- TUNING FORKS TESTS: RINNE TEST WEBER TEST 4- SCHWARTZE SIGN

1- OTITIS MEDIA 2- CONGENTIAL STAPES FIXATIONS 3- GONGENITAL MALLEAL HEAD FIXATION 4- TYMPANOSCLEROSIS 5- PAGET DISEASE 6- OSTEOGENESIS IMPERFECTA

1- CTSCAN: HALO SIGN

1- MEDICAL CARE A- SODIUM FLUORIDE 20 – 120 mg/D B- CALCIUM CARBONATE C- VITAMIN D EFFECTIVENESS: A- DISAPPEARANCE OF SCHWARTZE SIGN B.AUDIOMETRIC TESTS C. FOLLOW UP CT SCANNING 2-HEARING AIDS 3-SURGICAL CARE A- GENERAL ANEST. B-LOCAL ANEST. 1-STAPEDECTOMY 2-STAPEDETOMY

SNHL 1 – 2% PERMANENT FACIAL N. INJURY PROBABLY <1 PER 1000 CASES TYMPANIC MEMBRANE PERFORATIONS 1 – 2% ALTERATION OF TASTE DYSEQUILIBRIUM AND VERTIGO WITH NAUSEA AND VOMITING. TINNITUS

CHL 50 – 60 dB SNHL TOTAL DEAFNESS