The Inner Ear and Diseases of The Bony Labyrinth

3434 Part XXIX ◆ The Ear
Chapter 660
The Inner Ear and
Diseases of the Bony
Labyrinth
Joseph Haddad Jr and Sonam N. Dodhia
Genetic factors can impact the anatomy and function of the inner ear.
Infectious agents, including viruses, bacteria, and protozoa, also can
cause abnormal function, most commonly as sequelae of congenital
infection (see Table 655.2) or bacterial meningitis (see Chapter 621.1).
Other acquired diseases of the labyrinthine capsule include otosclerosis,
osteopetrosis, Langerhans cell histiocytosis (see Chapter 534.1), fibrous
dysplasia, and other types of bony dysplasia. All of these can cause both
conductive hearing loss (CHL) and sensorineural hearing loss (SNHL),
as well as vestibular dysfunction.
OTHER DISEASES OF THE INNER EAR

Labyrinthitis (also called vestibular neuritis) may be a complication
of direct spread of infection from acute or chronic otitis media or
mastoiditis and also can complicate bacterial meningitis as a result of
organisms entering the labyrinth through the internal auditory meatus,
endolymphatic duct, perilymphatic duct, vascular channels, or hema-
togenous spread. Clinical manifestations of vestibular neuritis can include
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3434.e2 Part XXIX ◆ The Ear
Keywords
Labyrinthitis
osteogenesis imperfecta
osteopetrosis
paroxysmal vertigo
vestibular migraine
vestibular neuritis
a sudden onset of rotatory vertigo, dysequilibrium, postural imbalance the hearing loss is severe enough, a hearing aid may be a preferable
(furniture walking) with falls to the affected side, deep-seated ear pain, alternative to surgical correction of the fixed stapes, because stapedectomy
nausea, vomiting, and spontaneous horizontal (occasionally rotary) in children with osteogenesis imperfecta can be technically very difficult,
nystagmus. and the disease and the hearing loss may be progressive.
The dizziness may last a few days, but balance issues, particularly Osteopetrosis, a very uncommon skeletal dysplasia, can involve the
following rapid head movements toward the affected ear, may last for temporal bone, including the middle ear and ossicles, resulting in a
months. Vestibular neuritis is usually unilateral and is not associated moderate to severe, usually CHL. Recurrent facial nerve paralysis also
with other neurologic defects; subjective hearing loss is unusual in can occur as a result of excess bone deposition; with each recurrence,
vestibular neuritis. If hearing loss is present, idiopathic SNHL should less facial function might return (see Chapter 719).
be considered, as well as classical labyrinthitis (vestibular and cochlear
nerves). Treatment of vestibular neuritis may include prednisone and Bibliography is available at Expert Consult.
vestibular rehabilitative exercises. Recurrent episodes should suggest
another diagnosis such as vestibular migraine or benign paroxysmal
positional vertigo.
In children, viral labyrinthitis is often associated with hearing loss.
Acute serous labyrinthitis, characterized by mild symptoms of vertigo
and hearing loss, most commonly develops secondary to middle-ear
infection without direct invasion. Acute suppurative labyrinthitis,
characterized by abrupt, severe onset of these symptoms, may be caused
by bacterial meningitis, or acute middle ear or mastoid infection via a
dehiscent horizontal semicircular canal. In these latter cases, a choles-
teatoma is almost always present. Treatment of acute infectious laby-
rinthitis includes antimicrobial agents in cases of bacterial infection or
antiviral agents (acyclovir, valacyclovir) in cases of herpes zoster oticus.
Oral corticosteroids reduce labyrinthine inflammation and may prevent
sequelae. A short course (≤3 days) of vestibular suppressants (dimen-
hydrinate 1-2 mg/kg) alleviates acute symptoms such as nausea. If it is
secondary to otitis media, otologic surgery may be required to remove
underlying cholesteatoma or drain the middle ear and mastoid. Chronic
labyrinthitis, most commonly associated with cholesteatoma, manifests
with SNHL and vestibular dysfunction that develops over time; surgery
is required to remove the cholesteatoma. Chronic labyrinthitis also
occurs uncommonly secondary to long-standing otitis media, with the
slow development of SNHL, usually starting in the higher frequencies,
and possibly with vestibular dysfunction. In addition, and more com-
monly, children with chronic middle-ear fluid often are unsteady or off
balance, a situation that improves immediately when the fluid resolves.
Vertigo and dizziness are common among older children and ado-
lescents. Benign paroxysmal vertigo, the most common cause of vertigo
in pediatric patients, is characterized by short periods of vertigo or
dizziness lasting seconds to a few minutes and associated with imbalance
and nystagmus; tinnitus or hearing loss is unusual. Basilar/vestibular
migraine is a common cause of episodic vertigo or dizziness and is
associated with headache (50–70% of patients), rotary or to-and-fro
nystagmus, and sensitivity to noise and bright light (see Chapter 613.1).
Benign paroxysmal positional vertigo is less common in young children
and more common with increasing age into adulthood. Particles form
in the semicircular canals (canalolithiasis), most often the posterior
canal; symptoms occur with position changes of the head and may last
sec to min. Vertigo and nystagmus may be demonstrated by changing
position (sitting to lying down on the right or left). Treatment involves
canalith repositioning maneuvers to shift the debris from the canals
into the utricle.
Otosclerosis, an autosomal dominant disease that affects only the
temporal bones, causes abnormal bone growth that can result in fixation
of the stapes in the oval window, leading to progressive hearing loss.
In one series in North America, otosclerosis was found in 0.6% of
temporal bones of children younger than 5 yr of age and 4% of those
ages 5-18 yr. The hearing loss is usually conductive at first, but SNHL
can develop. White girls and women are affected most commonly, with
onset of otosclerosis in teenagers or young adults, often associated
with pregnancy. Corrective surgery to replace the stapes with a mobile
prosthesis often is successful.
Osteogenesis imperfecta is a systemic disease that can involve both
the middle and inner ears (see Chapter 721). Hearing loss occurs in
approximately 20% of young children and as many as 50% of adults by
the age of 50 with this disease. The hearing loss most commonly is
conductive but can be sensorineural or mixed. Etiologies of hearing
loss include otosclerosis, ossicle fractures, or neural degeneration. If
Chapter 660 ◆ The Inner Ear and Diseases of the Bony Labyrinth 3435.e1
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lar disorders in children: a review, Int J Pediatr Otorhinolaryngol 78(5):718–724, 2014. infectious diseases, vol 1, 2014, Oxford University Press. S291-S291.
Goudakos JK, Markou KD, Psillas G, et al: Corticosteroids and vestibular exercises in Rodenburg-Vlot MBA, Ruytjens L, Oostenbrink R, et al: Systematic review: incidence
vestibular neuritis single-blind randomized clinical trial, JAMA Otolaryngol Head and course of hearing loss caused by bacterial meningitis: in search of an optimal
Neck Surg 140:434–440, 2014. timed audiological follow-up, Otol Neurotol 37(1):1–8, 2016.
Hamilton ST, Zuylen W, Shand A, et al: Prevention of congenital cytomegalovirus Rosenthal LS, Fowler KB, Boppana SB, et al: Cytomegalovirus shedding and delayed
complications by maternal and neonatal treatments: a systematic review, Rev Med sensorineural hearing loss: results from longitudinal follow-up of children with
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Jahn K, Langhagen T, Schroeder AS: Heinin: vertigo and dizziness in childhood—update Shao M, Xu P, Liu J, et al: The role of adjunctive dexamethasone in the treatment of
on diagnosis and treatment, Neuropediatrics 42:129–134, 2011. bacterial meningitis: an updated systematic meta-analysis, Patient Prefer Adherence.
Lawhn-Heath C, Buckle C, Christoforidis G, Straus C: Utility of head CT in the evalua- 10:1243, 2016.
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3434 Part XXIX ◆ The Ear

OTHER DISEASES OF THE INNER EAR

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