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The ear is divided anatomically into three sections (external, middle, and inner), and pathology contributing to hearing loss may strike one or more sections. Leading causes of conductive hearing loss include cerumen impaction, otitis media, and otosclerosis. Leading causes of sensorineural hearing loss include inherited disorders, noise exposure, and presbycusis. An understanding of the indications for medical management, surgical treatment, and amplification can help the family physician provide more effective care for these patients.
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The differential diagnosis of hearing loss can be simplified by considering the three major categories of loss.
Conductive hearing loss occurs when sound conduction is impeded through the external ear, the middle ear, or both.
Sensorineural hearing loss occurs when there is a problem within the cochlea or the neural pathway to the auditory cortex.
Mixed hearing loss is concomitant conductive and sensorineural loss.EvaluationA thorough history and a careful physical examination are essential to the diagnosis and treatment of hearing loss. An otoscope should be used to examine the external auditory canal for cerumen, foreign bodies, and abnormalities of the canal skin. The mobility, color, and surface anatomy of the tympanic membrane should be determined (Figure 1).


The sound remains midline in patients with normal hearing.The Rinne test compares air conduction with bone conduction. When the patient no longer can hear the sound, the tuning fork is placed adjacent to the ear canal (air conduction). In the presence of normal hearing or sensorineural hearing loss, air conduction is better than bone conduction. Audiograms objectively measure hearing levels and compare them with standards adopted by the American National Standards Institute in 1969.1 Normal hearing levels are 20 dB or better across all frequencies.
The audiogram measures air conduction and bone conduction and presents them graphically across the hearing frequencies. Audiographically demonstrated conductive hearing loss results in the air line falling below the bone line, creating an air-bone gap.Speech testing should be performed using standard word lists.
The speech reception threshold is the sound level at which 50 percent of presented words are understood. The speech recognition score is the percentage of words understood at 40 dB above the speech reception threshold.Conductive Hearing LossEXTERNAL EARComplete occlusion of the ear canal by cerumen is a frequent cause of conductive hearing loss.
Warm water (body temperature) irrigation is a safe method of removing cerumen in patients who have no history of otitis media, perforation of the tympanic membrane, or otologic surgery. The distance to the tympanic membrane must be kept in mind, because otoscopes do not allow for depth perception. Aqueous-based preparations, including docusate sodium, sodium bicarbonate, and hydrogen peroxide, are effective cerumenolytics.2,3Foreign bodies in the external auditory canal also can cause unilateral conductive hearing loss. If the object is not impacted or hygrostatic, warm water irrigation probably should be attempted first.
If this approach is not effective, the foreign body can be removed with an instrument if the patient is cooperative. If the patient is uncooperative, removal in an operating room may be necessary.Otitis externa is an infection of the skin of the external auditory canal. Patients with otitis externa experience pain on manipulation of the pinna or tragus, and their ear canal is edematous and filled with infectious debris. The most common pathogens in otitis externa are Pseudomonas aeruginosa and Staphylococcus aureus.4 Treatment involves debridement of the canal, followed by the application of ototopical drops. In patients with severe otitis externa, a wick is placed in the ear for two to three days to ensure delivery of the medication. The conductive hearing loss resolves after the inflammation subsides.Exostoses and osteomas are benign bony growths of the external auditory canal that interfere with normal cerumen migration, leading to occlusion and conductive hearing loss. Osteomas are single and unilateral, and are found at the bony-cartilaginous junction (Figure 2). Sebaceous cysts, fibromas, papillomas, adenomas, sarcomas, carcinomas, and melanomas also have been reported. If a malignancy is suspected, prompt biopsy is indicated.MIDDLE EARMiddle ear pathology may lead to conductive hearing loss.
Perforations of the tympanic membrane cause hearing loss by reducing the surface area available for sound transmission to the ossicular chain (Figure 3). In patients who have had chronic otitis media with tympanic membrane perforation, otoscopic examination and debridement are essential. Ototopical antibiotics (ofloxacin [Floxin]) are necessary, and oral antibiotics may be helpful.
Small perforations (less than 2 mm) often heal spontaneously.5 In the acute setting, blood may obstruct the ear canal and prevent visualization of the membrane.
If the perforation or hearing loss persists beyond two months, the patient should be referred for consideration of surgical correction. The diagnosis of otitis media can be confirmed by tympanometry and audiometry, and resolution of the effusion restores hearing.
Congenital cholesteatoma presents as a pearly white mass located behind an intact tympanic membrane in a patient with unilateral conductive hearing loss. Acquired cholesteatoma results from a retracted or perforated tympanic membrane with an ingrowth of epithelium. Conductive hearing loss caused by ossicular erosion is present in 90 percent of patients with cholesteatomas.8 Longstanding cholesteatomas expand to involve the mastoid, inner ear, and facial nerve.
Suspicion of cholesteatoma warrants surgical consultation.Myringosclerosis of the tympanic membrane develops in response to infection or inflammation (Figure 3). Irregular white patches consisting of calcium are visible on the membrane.9 Isolated myringosclerosis of the tympanic membrane rarely causes significant conductive hearing loss. However, extensive myringosclerosis, referred to as tympanosclerosis, involves the tympanic membrane, ossicular chain, and middle ear mucosa, and causes significant conductive hearing loss by stiffening the entire system.Otosclerosis is characterized by abnormal bone deposition at the footplate (base of stapes). This bone deposition leads to fixation of the stapes at the oval window, preventing normal vibration. Otosclerosis typically presents as progressive bilateral conductive hearing loss in middle-aged white women. It is the leading cause of conductive hearing loss in adults who do not have a middle ear effusion or a history of otitis media.10 There is usually a positive family history.


These neuroendocrine tumors arise from the adventitia of the jugular bulb or the neural plexus within the middle ear space. Characteristically, patients presenting with glomus tumors are women 40 to 50 years of age who report pulsatile tinnitus and hearing loss. On examination, a pulsating reddish-blue mass may be seen behind an intact tympanic membrane.
However, diagnosis of these tumors is difficult, and computed tomography of the temporal bones is required. Although most patients with this type of hearing loss are adults, children also can be affected. Hereditary and non-hereditary congenital hearing loss are the two major pediatric classifications.
The majority of hereditary losses are autosomal recessive and are frequently associated with other systemic findings. More than 100 congenital syndromes are associated with sensorineural hearing loss.The consequences of delayed detection can be significant.
Neonates considered at high risk for congenital hearing loss (Table 3)11 traditionally have been screened, and 30 states now require universal newborn auditory screening.12 The American Academy of Pediatrics and several other organizations endorse universal auditory screening. Year 2000 position statement: principles and guidelines for early hearing detection and intervention programs. The etiology is a combination of inherited and environmental factors, including lifetime noise exposure and tobacco use.
High frequencies are affected first, typically at 4,000 Hz, followed by middle and lower frequencies. The use of foam-insert earplugs decreases noise exposure by 30 dB.A less common cause of hearing loss is ototoxin exposure, typically from diuretics, salicylates, aminoglycosides, and many chemotheraupetic agents.
These medications must be administered carefully in patients who are elderly, have poor renal function, require a prolonged course of medication, or require simultaneous administration of multiple ototoxic agents. Patients with ototoxin exposure may experience hearing loss or dizziness.Autoimmune hearing loss has been diagnosed with increasing frequency since the 1980s.
Patients present with rapidly progressive bilateral sensorineural hearing loss and poor speech discrimination scores, and they also may have vertigo or disequilibrium.
Hearing loss progresses over three to four months, and an associated autoimmune disorder may be present. Symptoms usually improve with the administration of oral prednisone, and response to this steroid is currently the best way to make the diagnosis. Low-dose methotrexate therapy is becoming an accepted alternative to long-term prednisone therapy.14UNILATERAL HEARING LOSSTemporal bone fractures can cause unilateral sensorineural and conductive hearing loss. When the fracture line involves the bony labyrinth (cochlea or vestibule), sensorineural hearing loss occurs. Temporal bone injuries are associated with facial nerve paralysis, cerebrospinal fluid leakage, and other intracranial injuries. Early consultation is essential, and prompt surgical intervention may be required.Trauma may cause rupture of the round or oval window membranes, with perilymph leaking into the middle ear (fistula). Perilymph fistulas also may occur after straining, lifting, coughing, or sneezing, and are managed with three to six weeks of bed rest, followed by surgical repair if symptoms do not improve.15Meniere's disease is another cause of sensorineural hearing loss. Patients report unilateral fluctuating hearing loss with aural fullness, tinnitus, and episodic vertigo. Initially, the hearing loss is in the low frequencies, but higher frequencies are affected as the disease progresses.
The etiology of Meniere's disease remains unclear, but endolymphatic hydrops (increased fluid pressure within the inner ear) has been identified. The work-up consists of serial audiometry to document a fluctuating loss, vestibular testing to verify the diseased ear, and radiographic imaging to rule out an acoustic tumor. Hearing aids are often ineffective because patients suffer from poor speech discrimination, as well as diminished tolerance to amplified sound.
A history of upper respiratory infection within a month of the hearing loss is often described in patients who have a viral etiology.
The work-up includes audiometry, followed by radiographic imaging to rule out an acoustic tumor.
One study found that patients with minimal hearing loss, no vestibular symptoms, and early treatment have better outcomes.18Patients with acoustic neuromas present with unilateral sensorineural hearing loss approximately 10 to 22 percent of the time19 (Figure 6). Patients with asymmetric sensorineural hearing loss require evaluation for a retrocochlear tumor.



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