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H. Maraş Genç et al.
29 HIV- negative patients with cryptococcal meningitis, 73% had HL, and 90% were
sensorineural [130]. Among patients with SNHL, the severity of HL was mild or
moderate in the majority of patients. Hearing improved at 43% and stabilized at
38%. Internal auditory canal enhancement on MRI was associated with signicantly
more HL.Routine hearing surveillance in patients with cryptococcal meningitis was
recommended [130].
25.14 Autoimmune Encephalitis
Hearing loss in the course of autoimmune encephalitis is rarely reported and may
result from the involvement of the organ of Corti and/or the cochlear nuclei in the
brainstem [131]. Hearing loss is usually associated with the radiological involvement of the temporal lobe and/or brainstem in reported cases. It can be the presenting symptom in some patients [132–134].
In a cohort of paraneoplastic autoimmune Kelch-like protein-11 IgG seropositive
cases, HL was present in 15/39 (39%) patients [134]. Radiologically, the temporal
lobes, brainstem, and cerebellum were involved. The disease typically presented
with rhombencephalitis, with clinical ndings, including ataxia, diplopia, dysarthria, vertigo, HL, and tinnitus. Hearing loss was refractory to treatment. In an adult
patient, bilateral HL was the initial symptom of anti-NMDAR encephalitis and signicantly improved after immunotherapy [133].
25.15 Conclusion
Hearing loss can be seen after meningoencephalitis, although it is not as common as
in meningitis. Hearing loss may be independent of or associated with encephalitis;
however, it is usually more severe if the latter is the case. Hearing loss can occur in
the acute phase of the disease, during recovery, or years later. In children, TORCH
infections are a signicant cause of HL, and the result of neonatal hearing screening
may be normal in most of these patients. Early diagnosis and specic treatment of
the offending pathogen may change the prognosis. Common childhood vaccines
can prevent infections, including mumps, measles, rubella, and varicella, important
causes of HL.Periodic and long-term follow-up is recommended in patients with
infections that cause HL.
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Part IV
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Bacterial Infections
Bacterial Infections inChildren
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andHearing Loss: AnOverview
AhmetSoysal, EminSamiArısoy, andArmandoG.Correa
26.1 Introduction
A person who cannot hear and someone with normal hearing, with hearing thresholds of 20 decibels (dB) or better in both ears, is diagnosed as having hearing loss
(HL). Hearing loss may be mild, moderate, severe, or profound. It can affect one or
both ears leading to difculty hearing conversational speech or loud sounds [1].
“Hard of hearing” refers to people with HL ranging from mild to severe. People
with hearing difculties usually communicate through spoken language and benet
from hearing aids, cochlear implants, and other assistive devices and captioning.
“Deaf” people mostly have profound HL, implying little or no hearing. They often
use sign language for communication.
The World Health Organization (WHO) reported that over 5% of the world’s
population, 432 million adults and 34 million children, have HL [1]. By 2050, over
700 million people, one in every 10 people, will have disabling HL. “Disabling” HL
26
A. Soysal (*)
Section of Pediatric Infectious Diseases, Memorial Ataşehir Hospital, İstanbul, Türkiye
e-mail: drahmetsoysal20@gmail.com
E. S. Arısoy
Division of Pediatric Infectious Diseases, Department of Pediatrics, Faculty of Medicine,
Kocaeli University, Kocaeli, Türkiye
e-mail: emin.sami.arisoy@gmail.com
A. G. Correa
Division of Academic General Pediatrics, Department of Pediatrics, Baylor College of
Medicine, Houston, TX, USA
Section of International and Destination Medicine, Texas Children’s Hospital,
Houston, TX, USA
e-mail: acorrea@bcm.edu
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2023
A. E. Arısoy et al. (eds.), Hearing Loss in Congenital, Neonatal and Childhood
Infections, Comprehensive ENT, https://doi.org/10.1007/978-3-031-38495-0_26
389
390
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refers to HL greater than 35dB in the better-hearing ear. Nearly 80% of people with
disabling HL live in low- and middle-income countries. The prevalence of HL
increases with age; among those older than 60years, over 25% are affected by disabling HL [1]. Early identication of HL in early childhood is critical since HL in
the rst year of life can lead to delays in speech, language, and cognitive development [2]. Those developmental delays secondary to hearing problems are usually
retainable.
Nearly 2–3 out of every 1000 children in the United States of America (USA) are
born with a detectable level of HL in one or both ears [3], and among deaf children,
more than 90% of them are born to normal hearing parents [4]. Before introducing
the pneumococcal conjugate vaccine, nearly ve out of six children experienced
middle ear infections by the age of 3years; almost all children developed transient
HL related to middle ear infections from birth to 11years of age [5, 6].
A. Soysal et al.
26.2 Mechanisms andCauses ofHearing Loss
Hearing loss in children may occur during intrauterine life and after birth. Hearing
loss can be categorized into three groups according to the mechanisms of hearing
impairment.
26.2.1 Conductive Hearing Loss
Conductive HL is caused by a problem in the outer or middle ear that interferes with
sound conduction to the inner ear. It can occur from the outer parts of the ear (pinna,
external auditory canal) to the stapes footplate and oval window. In children, conductive HL is most often transient (e.g., otitis media with effusion), but it can be
permanent (e.g., aural atresia or chronic adhesive otitis media).
Two infections are among the most common causes of conductive HL in children. The rst is otitis externa, which develops after local trauma to the ear canal or
when impacted cerumen becomes contaminated by bacteria after swimming or
showering. Hearing loss may occur if there is a considerable accumulation of debris,
edema, or inammation in the ear canal.
Acute otitis media (AOM) is the other most common childhood disorder associated with conductive HL.By 3years, most children will experience at least one
episode of AOM, and many will have experienced at least three episodes. Acute
otitis media leads to uid accumulation in the middle ear space that prevents tympanic membrane vibration, thereby diminishing the movement of the ossicular
chain. Hearing loss persists until the resorption of uid from the middle ear space.
Middle ear effusion can last 6weeks despite adequate therapy in many patients. The
uid accumulation, also one of the causes of conductive HL, sometimes leads to
tympanic membrane perforation,
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