A 28 year female patient presented with history of bilateral hearing loss and tinnitus. She can hear better in noisy environment. Examination showed intract ear drums bilaterally and Rinne test is negative bilaterally. Pure tone audiometry given below. What is the most probable diagnosis?
Ans. (c) Stapedial otosclerosisRef: Logan Turners ENT 11th Ed; Page No-464Otosclerosis* Most commonly seen in third decade, i.e. 20-30 years of age.* Most commonly seen in females, with a ratio of 2:1.* It is most commonly seen in white peoples.* The most common site origin of otosclerosis is fissula ante fenestrum.* It is aggravated by any hormonal change, be it pregnancy or menopause.* It is an autosomal dominant disorder and positive family history.* Clinical features:# Patients presents with bilateral progressive conductive hearing loss (70-85%).# Otosclerosis is the most common cause of bilateral progressive conductive hearing loss in adults.# This patient gives the typical history of haring better in noisy surroundings, which is known as Paracusis willisii phenomenon. This is because the speaker raises his voice in noisy surroundings which now cross the hearings threshold of the patient.# Less commonly tinnitus can also occur in otosclerosis, particular in cochlear otosclerosis and active otosclerosis.# Sometimes otosclerosis is associated with Osteogenesis imperfecta and blue sclera, when it constitutes van dar Hoeve syndrome.* Investigation:# Tympanic membrane appears pearly white (normal) in 90% patients; it is called as mature otosclerosis.# 10% cases of otosclerosis are active. In these the active division is taking place on the medial wall of middle ear leading to increase vascularity. This appears pinkish through the intact tympanic membrane leading to flamingo pink appears of TM also known as Schwartz sign.# Audiometric test:Tuning fork testFindingsRinne'sNegativeWeber'sLateralized to the worst earABCSame as examinerSchwabachLengthenedGelle'sNegative (i.e. change in the pressure of EAC does not produce ant y change in hearings as ossicular chain is already fixed.* Pure tone Audiometry:# The audiogram of the patients shows anA-B gap > 15dB (Suggestive of conductive hearing loss). Complete fixation of foot plate leads to the maximum conductive hearing loss of 60dB.# There is typical dip at 2000Hz in the bone conduction curve. This dip is because of fixation of footplate of stapes, the natural frequency of which 2000Hz. Hence its fixation fails to transit this frequency sound.# This dip is known as "Carhart's notch". This dip disappears after the mobilization of footplate and stapes during the management of otosclerosis.TreatmentActive casesMature cases* Sodium fluoride (NaF):# Inactive cases i.e. when Scwartze sign is present, medial medical management with sodium fluoride is done.# NaF accelerates the maturation of the focus by increasing osteoblastic and decreasing osteolytic activity.# It also inhibits the proteolytic enzymes that release cytotoxic chemicals to Cochlea.* Surgery: In mature otosclerosis when the foot plate is fixed and active division has stopped the treatment consists of mobilization of foot plate of stapes. The surgical procedure s are:-* Stapedectomy:# Here the whole of the stapes is removed and the oval window is then covered with a fascia graft and prosthesis is placed over the graft with other end of prosthesis attached to the long process of incus.# The change of perilymph fistula, SN hearing loss and vertigo is higher in stapedectomy as compared to stapedotomy. Hence it is not preferred nowadays.# It is contraindicated in chronic nephritis, RA, pregnancy and lactation.* Bisphosphonates have also been used as they inhibit bone resorption.* Stapedotomy:# This is a more conservative and better procedure where the incudostapedial joint is separated; the Stapedial tendon is cut and the suprastructure of stapes (head, neck, anterior and posterior crura) is removed leaving the foot plate behind.# The most common prosthesis is used here Teflon piston.* Postoperative Complication:# Conductive hair loss.# Perilymph fistula# Facial nerve palsyHow to rule out other options?Meniere's disease# It is also known as endolymphatic hydrops. It is frequently a unilateral condition and seen in more commonly in young females (20-40 years).# It is largely idiopathic.Clinical features* Vertigo: It is the first symptoms of Meniere's disease and it lasts for at least 20 minutes till a maximum of up to 24 hours. This sudden onset of vertigo of Meniere's disease associated with vagal symptoms, i.e. nausea, vomiting, abdominal cramping, diarrhoea and bradycardia.* SN hearing loss and tinnitus can be seen.Investigations* Rinne Test: Positive* Weber test: Towards the normal ear* ABC: Shortened* Schwabach: Shortened* Recruitment: Positive* SISIS:70-100% (because of recruitment)Management* Salt and water restrictions* Labyrinthine sedatives* Vasodilators* Meniett deviceVestibular neuronitis* This condition is characterized by acute onset vertigo, nystagmus and tinnitus with nausea and vomiting lasting for days to weeks.* This is most likely because of viral infection involving the vestibular nave.* The cochlear part is not involved, so the hearing remains normal.* The caloric test shows diminished response.* It is managed by labyrinthine sedatives.
Meniere's Disease
Endolymphatic fistula
Stapedial otosclerosis
Vestibular schwannoma
Ear