Ann Otol 88 :1979
TEMPORAL BONE HISTOPATHOLOGY IN DEAFNESS DUE TO CRYPTOCOCCAL MENINGITIS T AKEHIKO
HARADA,
ISAMU SANDO, MD, DMS
MD
EUGENE N. MYERS, MD, FACS
PrrrSBURGH, PENNSYLVANIA
This paper reports on a patient who survived an attack of cryptococcal meningitis eight years prior to his death. A bilateral sensorineural hearing loss had been noted a short time oefore the patient was admitted to the hospital, and was the only complication after he recovered from the disease. Histopathologic study of the temporal bones showed a similar pattern of pathology in both ears, the most striking finding being a severe loss of spiral ganglion cells in Rosenthal's canal, and of cochlear nerve fibers in the osseous spiral lamina and internal auditory meatus. The vestibular nerve was mostly free from pathology. The organ of Corti was atrophic but the hair cell population appeared to be almost normal. A slight nwnber of cryptococci were observed in limited areas of the cochlear and the saccular nerves in the internal auditory meatus. The severe pathology of the cochlear nerve was compatible with audiologic evaluations, which pointed to a retrocochlear lesion. Thus, this case demonstrates some characteristic aspects of cryptococcal infection of the temporal bone: The primary site of invasion was the cochlear nerve in the internal auditory meatus and the modiolus, leading to the loss of ganglion cells and nerve fibers, while the vestibular nerve appears to have been resistant to infection.
Cryptococcus neoformans, an encapsulated, budding, yeast-like fungus which is found in soil and pigeon nests, has a special predilection for the central nervous system. Cryptococcosis, an infection caused by C. neoformans, is usually established in the lung and other viscera before dissemination to the brain and meninges occurs. It occurs with increased frequency in patients with leukemia or lymphoma.' The meningitis associated with C. neoformans, the bestknown form of the disease, was first reported by Verse" in 1914, and the disease had been known to be almost always fatal until the advent of amphotericin B, which improved the prognosis.
In 1975, Igarashi, et ala first reported the temporal bone findings of a patient who died at the fulminant stage of cryptococcal meningitis. They indicated that the primary site of the invasion in the temporal bones was within the neural system. This finding was subsequently confirmed in a report made by McGilJ.4 The purpose of this paper is to present the findings of the temporal bones
of a patient who, eight years prior to his death, had cryptococcal meningitis from which he made an almost complete recovery with hearing loss as the only residual complication. Although this case is, to our knowledge, the third report on temporal bone histopathology associated with cryptococcal meningitis, this is the only one which shows the pathology remaining after recovery from the disease. CASE REPORT
A 55-year-old male was admitted to Presbyterian-University Hospital of Pittsburgh on May 27, 1965, with marked confusion, staggering gait, and generalized nonfocal seizures associated with urinary incontinence, which had developed several days prior to his admission. For several weeks before his admission he had noted a bilateral hearing loss with occasional tinnitus, but did not experience vertigo. The patient had been a construction worker for 32 years, and had been suffering from chronic lymphocytic leukemia for 9 years, but had never had trouble with his hearing before this time.
From the Departments of Otolaryngology and Pathology, University of Pittsburgh School of Medicine and the Eye and Ear Hospital of Pittsburgh, Pittsburgh, Pennsylvania. This study was supported by NIH Grant 5-R01-NS13787-02.
630
Downloaded from aor.sagepub.com at UNIV OF PENNSYLVANIA on June 16, 2015
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TEMPORAL BONE HISTOPATHOLOGY IN DEAFNESS DUE TO CRYPTOCOCCAL MENINGITIS T AKEHIKO
HARADA,
ISAMU SANDO, MD, DMS
MD
EUGENE N. MYERS, MD, FACS
PrrrSBURGH, PENNSYLVANIA
This paper reports on a patient who survived an attack of cryptococcal meningitis eight years prior to his death. A bilateral sensorineural hearing loss had been noted a short time oefore the patient was admitted to the hospital, and was the only complication after he recovered from the disease. Histopathologic study of the temporal bones showed a similar pattern of pathology in both ears, the most striking finding being a severe loss of spiral ganglion cells in Rosenthal's canal, and of cochlear nerve fibers in the osseous spiral lamina and internal auditory meatus. The vestibular nerve was mostly free from pathology. The organ of Corti was atrophic but the hair cell population appeared to be almost normal. A slight nwnber of cryptococci were observed in limited areas of the cochlear and the saccular nerves in the internal auditory meatus. The severe pathology of the cochlear nerve was compatible with audiologic evaluations, which pointed to a retrocochlear lesion. Thus, this case demonstrates some characteristic aspects of cryptococcal infection of the temporal bone: The primary site of invasion was the cochlear nerve in the internal auditory meatus and the modiolus, leading to the loss of ganglion cells and nerve fibers, while the vestibular nerve appears to have been resistant to infection.
Cryptococcus neoformans, an encapsulated, budding, yeast-like fungus which is found in soil and pigeon nests, has a special predilection for the central nervous system. Cryptococcosis, an infection caused by C. neoformans, is usually established in the lung and other viscera before dissemination to the brain and meninges occurs. It occurs with increased frequency in patients with leukemia or lymphoma.' The meningitis associated with C. neoformans, the bestknown form of the disease, was first reported by Verse" in 1914, and the disease had been known to be almost always fatal until the advent of amphotericin B, which improved the prognosis.
In 1975, Igarashi, et ala first reported the temporal bone findings of a patient who died at the fulminant stage of cryptococcal meningitis. They indicated that the primary site of the invasion in the temporal bones was within the neural system. This finding was subsequently confirmed in a report made by McGilJ.4 The purpose of this paper is to present the findings of the temporal bones
of a patient who, eight years prior to his death, had cryptococcal meningitis from which he made an almost complete recovery with hearing loss as the only residual complication. Although this case is, to our knowledge, the third report on temporal bone histopathology associated with cryptococcal meningitis, this is the only one which shows the pathology remaining after recovery from the disease. CASE REPORT
A 55-year-old male was admitted to Presbyterian-University Hospital of Pittsburgh on May 27, 1965, with marked confusion, staggering gait, and generalized nonfocal seizures associated with urinary incontinence, which had developed several days prior to his admission. For several weeks before his admission he had noted a bilateral hearing loss with occasional tinnitus, but did not experience vertigo. The patient had been a construction worker for 32 years, and had been suffering from chronic lymphocytic leukemia for 9 years, but had never had trouble with his hearing before this time.
From the Departments of Otolaryngology and Pathology, University of Pittsburgh School of Medicine and the Eye and Ear Hospital of Pittsburgh, Pittsburgh, Pennsylvania. This study was supported by NIH Grant 5-R01-NS13787-02.
630
Downloaded from aor.sagepub.com at UNIV OF PENNSYLVANIA on June 16, 2015
CRYPTOCOCCAL MENINGITIS
250
PURE TONE AUDIOGRAM 500 1000 2000 4000 150
1500
250
8000
750
--
-..: > -c
"'til '"
..J
I
o
0
'"
0
··
0
0
,~ bf
-"
40
u
5
0
0
0
0
'" " '~"
100
OIl" 4-
