Congenital Aural Atresia — MCQs
In which condition do symptoms improve with crying?
A 45-year-old gentleman reports decreased hearing in the right ear for the last two years. On testing with a 512 Hz tuning fork, the Rinne's test without masking is negative on the right ear and positive on the left ear. With the Weber's test, the tone is perceived as louder in the left ear. Patient most likely has -
Which branchial arches are associated with Goldenhar syndrome?
Surgery to widen the cartilaginous part of EAC
Which of the following is used for initial screening of auditory function in a neonate?
Which of the following interventions is least appropriate for an 8-year-old boy with bilateral sensorineural hearing loss?
The treatment of choice for atticoantral variety of chronic suppurative otitis media is:
What is a specific indication for the use of a Bone-Anchored Hearing Aid (BAHA)?
The cough response elicited while cleaning the external ear canal is mediated by stimulation of which nerve?
Which statement is not true regarding otosclerosis?
Congenital Aural Atresia Indian Medical PG Practice Questions and MCQs
Question 1: In which condition do symptoms improve with crying?
- A. Tetralogy of Fallot
- B. Choanal atresia (Correct Answer)
- C. Bronchial asthma
- D. None of the options
Explanation: ***Choanal atresia*** - In **bilateral choanal atresia**, a newborn is unable to breathe through the nose due to a bony or membranous obstruction. Symptoms like **cyanosis** worsen with feeding and improve with crying because crying involves **mouth breathing**, which bypasses the nasal obstruction. - This condition is a **medical emergency** as newborns are obligate nasal breathers, and immediate intervention (e.g., oral airway, surgical repair) is often required. *Tetralogy of Fallot* - This congenital heart defect can lead to **cyanotic spells** (hypercyanotic or "tet" spells), which are characterized by increased cyanosis, tachypnea, and irritability. These spells are often precipitated by activities that increase right-to-left shunting, and **crying can worsen them** rather than improve them due to increased systemic oxygen demand and vascular resistance. - Management often involves positioning (knee-chest) and medications to reduce pulmonary vascular resistance or increase systemic vascular resistance, to alleviate shunting. *Bronchial asthma* - **Crying is a common trigger for asthma attacks** in children, as it can induce bronchoconstriction due to hyperventilation and airway irritation. Symptoms typically worsen with crying, leading to **wheezing, coughing, and shortness of breath**. - Asthma is characterized by **reversible airway obstruction** and airway hyperresponsiveness, and its symptoms do not improve with crying. *None of the options* - This option is incorrect because **choanal atresia** is a condition where symptoms (specifically cyanosis) do improve with crying due to the switch from nasal to mouth breathing.
Question 2: A 45-year-old gentleman reports decreased hearing in the right ear for the last two years. On testing with a 512 Hz tuning fork, the Rinne's test without masking is negative on the right ear and positive on the left ear. With the Weber's test, the tone is perceived as louder in the left ear. Patient most likely has -
- A. Right conductive hearing loss (Correct Answer)
- B. Right sensorineural hearing loss
- C. Left sensorineural hearing loss
- D. Left conductive hearing loss
Explanation: ***Right conductive hearing loss*** - A **negative Rinne's test** (bone conduction louder than air conduction) in the right ear indicates **conductive hearing loss** on that side - However, in true conductive hearing loss, **Weber should lateralize to the affected (right) ear** due to the occlusion effect, not to the left ear - The Weber lateralizing to the left ear with a negative Rinne on the right suggests this may be a **false negative Rinne** due to lack of masking, where sound crosses over to the better left ear - This combination is atypical for pure conductive loss and requires repeat testing with proper masking *Right sensorineural hearing loss* - In **sensorineural hearing loss**, Rinne's test should be **positive** (air conduction > bone conduction) on both sides, though both may be reduced on the affected side - **Weber lateralizes to the unaffected (left) ear**, which matches the given finding - The **negative Rinne on the right ear without masking** is likely a **false negative** due to sound crossing over to the better left ear during bone conduction testing - This is the **most consistent interpretation** when Rinne testing is done without masking, but traditionally the question frame suggests conductive loss *Left sensorineural hearing loss* - Would show **positive Rinne bilaterally** with reduced hearing on the left - **Weber would lateralize to the right ear** (the better ear), contradicting the given findings - This option is clearly inconsistent with the clinical findings *Left conductive hearing loss* - Would show **negative Rinne on the left** and positive on the right - Weber would lateralize to the left ear (affected side in conductive loss) - The **Rinne findings contradict this**, as the right ear shows negative Rinne, not the left
Question 3: Which branchial arches are associated with Goldenhar syndrome?
- A. 1st branchial arch
- B. 1st & 2nd branchial arch (Correct Answer)
- C. 2nd & 3rd branchial arch
- D. 3rd & 4th branchial arch
Explanation: ***1st & 2nd branchial arch*** - **Goldenhar syndrome** (oculo-auriculo-vertebral spectrum) is a congenital condition characterized by developmental abnormalities affecting structures derived from the **first and second branchial arches**. - This typically includes abnormalities of the **ear, eye, and spine**, reflecting the developmental origin of these structures. *1st branchial arch* - While the first branchial arch is involved in Goldenhar syndrome, the syndrome's presentation extends to structures derived from the **second branchial arch** as well. - Anomalies limited solely to the first arch structures (mandibular and maxillary prominences) would not encompass the full spectrum of Goldenhar syndrome. *2nd & 3rd branchial arch* - The second branchial arch is affected in Goldenhar syndrome, but the **third branchial arch** is generally not primarily associated with the typical features of this condition. - The third arch contributes to the **hyoid bone** and parts of the pharynx and tongue, which are not hallmark features of Goldenhar syndrome. *3rd & 4th branchial arch* - Neither the **third nor the fourth branchial arches** are primarily implicated in the pathogenesis of Goldenhar syndrome. - The fourth branchial arch forms parts of the **larynx and pharyngeal muscles**, and its involvement would point to different congenital anomalies.
Question 4: Surgery to widen the cartilaginous part of EAC
- A. Tympanoplasty
- B. Otoplasty
- C. Myringoplasty
- D. Meatoplasty (Correct Answer)
Explanation: ***Meatoplasty*** - A **meatoplasty** involves enlarging the external auditory meatus, which is the cartilaginous opening of the external auditory canal (EAC). - This procedure is often performed to improve **ventilation** and **drainage**, or to facilitate the fitting of hearing aids. *Tympanoplasty* - A **tympanoplasty** is a surgical procedure to repair the **tympanic membrane (eardrum)** and/or the **ossicular chain**. - Its primary goal is to improve hearing and prevent recurrent ear infections, not to widen the EAC. *Otoplasty* - **Otoplasty** is a cosmetic surgical procedure to reshape the **external ear (pinna)**, often to correct prominent ears. - It does not involve modifying the external auditory canal. *Myringoplasty* - **Myringoplasty** is a specific type of tympanoplasty focused solely on repairing a **perforated tympanic membrane**. - It does not involve widening the cartilaginous part of the EAC.
Question 5: Which of the following is used for initial screening of auditory function in a neonate?
- A. Otoacoustic emission (OAE) (Correct Answer)
- B. Auditory brainstem response (ABR)
- C. Pure tone audiometry (PTA)
- D. Free field audiometry
Explanation: ***Otoacoustic emission (OAE)*** - **OAE** is the **gold standard for universal newborn hearing screening (UNHS)** programs worldwide due to its **non-invasive nature**, speed, and cost-effectiveness. - The test measures **sound waves produced by the outer hair cells of the cochlea** in response to auditory stimuli, indicating normal cochlear function. - **Quick to perform (2-3 minutes)**, requires minimal cooperation, and can be done while the infant is sleeping. *Auditory brainstem response (ABR)* - While **ABR** is a definitive diagnostic test for hearing loss, it is typically used as a **second-stage test** if an OAE screening fails, rather than the initial screening tool. - ABR measures the **brain's response to sound**, providing information about the neural pathway from the cochlea to the brainstem. - More **time-consuming and expensive** than OAE, making it less suitable for mass screening. *Pure tone audiometry (PTA)* - **PTA** requires active participation and understanding of instructions, making it **unsuitable for neonates** and young children. - This test is primarily used for **older children (typically >4 years) and adults** to determine hearing thresholds across various frequencies. *Free field audiometry* - **Free field audiometry** involves presenting sounds through loudspeakers to assess hearing, but it is **not suitable for precise threshold determination** in neonates due to their inability to localize sounds reliably or respond consistently. - It's mainly used for behavioral observation audiometry in older infants (6-24 months), but **not as a primary screening method** for neonates.
Question 6: Which of the following interventions is least appropriate for an 8-year-old boy with bilateral sensorineural hearing loss?
- A. Adenoidectomy with grommet insertion (Correct Answer)
- B. Hearing aid
- C. Cochlear implant
- D. Preferential sitting in the classroom
Explanation: ***Adenoidectomy with grommet insertion*** - This procedure addresses **conductive hearing loss** (e.g., due to **otitis media with effusion**), while the boy has **sensorineural hearing loss (SNHL)**. - **Grommet insertion** (tympanostomy tubes) is used to improve middle ear ventilation and drain fluid, which is irrelevant for SNHL. *Hearing aid* - A **hearing aid** amplifies sound and is a common and appropriate intervention for **sensorineural hearing loss**, especially for mild to severe cases. - It can significantly improve a child's ability to hear and develop speech. *Cochlear implant* - A **cochlear implant** is appropriate for children with **severe to profound sensorineural hearing loss** who do not benefit sufficiently from hearing aids. - It directly stimulates the auditory nerve, bypassing damaged parts of the inner ear. *Preferential sitting in the classroom* - This is a simple and effective **accommodative strategy** to improve a child's listening environment and is appropriate for any degree of hearing loss. - It helps the child to better hear the teacher and participate in classroom discussions.
Question 7: The treatment of choice for atticoantral variety of chronic suppurative otitis media is:
- A. Mastoidectomy (Correct Answer)
- B. Medical management
- C. Underlay myringoplasty
- D. Insertion of ventilation tube
Explanation: **Correct: Mastoidectomy** - The **atticoantral** variety of chronic suppurative otitis media (CSOM) is typically associated with **cholesteatoma**, which necessitates surgical eradication to prevent complications such as intracranial infection, facial nerve palsy, and labyrinthine destruction. - **Mastoidectomy** is the treatment of choice to remove the cholesteatoma and achieve a safe, dry ear by clearing disease from the mastoid air cells and attic. *Incorrect: Medical management* - This approach is typically used for the **tubotympanic** (mucosal/safe) type of CSOM, which involves a central perforation without cholesteatoma. - It is **ineffective in the presence of cholesteatoma**, as antibiotics cannot penetrate the keratinized debris matrix and do not eradicate the underlying pathology. *Incorrect: Underlay myringoplasty* - This procedure repairs a **tympanic membrane perforation** but does not address the underlying cholesteatoma or disease within the mastoid and attic. - It is used for **safe, dry perforations**, usually associated with the tubotympanic type of CSOM after the ear has been rendered inactive. *Incorrect: Insertion of ventilation tube* - Ventilation tubes (grommets) are primarily used for **recurrent acute otitis media** or **otitis media with effusion (glue ear)** to equalize middle ear pressure and facilitate drainage. - They are **not indicated for CSOM**, especially the atticoantral type with cholesteatoma, as they do not resolve the chronic infection or remove the pathological tissue.
Question 8: What is a specific indication for the use of a Bone-Anchored Hearing Aid (BAHA)?
- A. Bilateral conductive hearing loss
- B. Unilateral sensorineural hearing loss
- C. Congenital canal atresia (Correct Answer)
- D. Conductive hearing loss due to other causes
Explanation: ***Congenital canal atresia*** - **Congenital canal atresia** represents a classic, specific indication for BAHA because the ear canal is congenitally absent or severely stenosed, making conventional hearing aids impossible to fit or ineffective. - BAHA bypasses the absent ear canal entirely by transmitting sound vibrations directly to the **cochlea through bone conduction**, providing an ideal solution for this anatomical abnormality. *Bilateral conductive hearing loss* - While BAHA can be used for conductive hearing loss, **conventional hearing aids** are typically the first-line treatment when ear canals are intact and functional. - Bilateral cases often benefit from traditional amplification devices or **surgical correction** of the underlying conductive pathology before considering BAHA. *Unilateral sensorineural hearing loss* - BAHA is commonly used for **single-sided deafness (SSD)** to route sound from the deaf ear to the hearing ear via bone conduction using the **CROS principle**. - However, this indication is less specific than congenital canal atresia, as other treatment options like **conventional CROS hearing aids** may also be considered. *Conductive hearing loss due to other causes* - This option is too **broad and non-specific** as it encompasses many different etiologies of conductive hearing loss. - Many other causes of conductive hearing loss have **alternative treatments** such as surgical repair, conventional hearing aids, or medical management, making BAHA a secondary consideration.
Question 9: The cough response elicited while cleaning the external ear canal is mediated by stimulation of which nerve?
- A. The V cranial nerve
- B. Innervation of the external ear canal by C1 and C2
- C. The X cranial nerve (Correct Answer)
- D. Branches of the VII cranial nerve
Explanation: This phenomenon is known as **Arnold’s Reflex** (or the Ear-Cough Reflex). It occurs due to the stimulation of the **Auricular branch of the Vagus Nerve (Arnold’s Nerve)**, which provides sensory innervation to the posterior and inferior walls of the external auditory canal (EAC). ### Why the Correct Answer is Right: When the EAC is stimulated (e.g., during syringing or cleaning with a cotton bud), sensory impulses are carried via the Vagus nerve (CN X) to the nucleus tractus solitarius in the brainstem. This triggers the efferent limb of the cough reflex, leading to an involuntary cough. This is a classic example of a referred reflex where stimulation of a peripheral nerve causes a response in a visceral organ system. ### Why Other Options are Incorrect: * **Option A (V cranial nerve):** The Auriculotemporal branch of the Mandibular nerve (V3) supplies the anterior and superior walls of the EAC. While it carries sensation, it does not mediate the cough reflex. * **Option B (C1 and C2):** The Greater Auricular nerve (C2, C3) supplies the skin over the mastoid and the lateral/lower part of the auricle, not the deep canal associated with this reflex. * **Option C (VII cranial nerve):** The Facial nerve provides minor sensory innervation to the concha and retroauricular area (often involved in Ramsay Hunt Syndrome), but it is not responsible for the cough reflex. ### High-Yield Clinical Pearls for NEET-PG: * **Arnold’s Nerve:** A branch of the Vagus (CN X). * **Vagal Reflexes in ENT:** Stimulation of the EAC can occasionally cause **bradycardia or fainting** (Vaso-vagal syncope) due to the same nerve. * **Hitchelberger’s Sign:** Reduced sensation in the area supplied by the facial nerve in the EAC, seen in Acoustic Neuroma. * **Nerve Supply of EAC (Summary):** 1. Anterior/Superior: V3 (Auriculotemporal). 2. Posterior/Inferior: X (Arnold’s). 3. Concha/Posterior wall: VII (Facial).
Question 10: Which statement is not true regarding otosclerosis?
- A. Hearing improves in noisy environments (Carhart's notch)
- B. The Eustachian tube is always abnormal (Correct Answer)
- C. The tympanic membrane appears normal
- D. It causes bilateral progressive conductive deafness
Explanation: ### Explanation **Otosclerosis** is a primary metabolic bone disease of the otic capsule characterized by the replacement of normal bone with vascular spongy bone, leading to stapes fixation and conductive hearing loss. **Why Option B is the Correct Answer (The False Statement):** The pathology of otosclerosis is strictly limited to the **bony labyrinth** of the inner ear and the **stapes footplate**. It does not involve the middle ear mucosa or the nasopharynx. Therefore, the **Eustachian tube function remains perfectly normal**. Any middle ear pathology involving Eustachian tube dysfunction (like Serous Otitis Media) would be a separate clinical entity. **Analysis of Other Options:** * **Option A:** This refers to **Paracusis Willisi**, where patients hear better in noisy surroundings because others speak louder to overcome background noise. While the option mentions *Carhart’s notch* (a dip in bone conduction at 2000 Hz), it is important to note that Paracusis Willisi is the clinical phenomenon, while Carhart's notch is the characteristic audiometric finding. * **Option C:** In most cases of otosclerosis, the **tympanic membrane is normal and mobile**. A rare exception is "Schwartze sign" (a flamingo-pink flush seen through the drum), but the membrane itself remains structurally normal. * **Option D:** Otosclerosis typically presents as **bilateral (80% of cases)**, progressive conductive hearing loss, usually starting in the second or third decade of life. **High-Yield Clinical Pearls for NEET-PG:** 1. **Gelle’s Test:** Negative (indicates stapes fixation). 2. **Tympanometry:** Typically shows an **As type** curve (stiffened ossicular chain). 3. **Schwartze Sign:** Indicates active otosclerosis (Otospongiosis). 4. **Treatment of Choice:** **Stapedotomy** (using a Teflon piston). 5. **Medical Management:** Sodium Fluoride (to inhibit osteoclastic activity and mature the focus).
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