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Acute bilateral sensorineural hearing loss as the presenting symptom of metastatic lung cancer
Acute bilateral sensorineural hearing loss as the presenting symptom of metastatic lung cancer MARY C. SNYDER, MD, and BARBARA M. HEYWOOD, MD, Omaha, Nebraska
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eptomeningeal carcinomatosis is the multifocal spread of tumor cells to the leptomeninges from a distant solid tumor, most commonly adenocarcinoma of the breast or lung, or melanoma. The overwhelming majority of cases are diagnosed in patients with a known malignancy. Affected patients present with acute, multifocal, neurologic deficits. Vestibulocochlear nerve involvement often results in sensorineural hearing loss, tinnitus, and vertigo. Although considered a relatively rare disorder, leptomeningeal carcinomatosis is being diagnosed more frequently as improved treatments provide cancer patients with longer survival times.1 We present a case of acute hearing loss as a result of leptomeningeal carcinomatosis in a patient with a previously undiagnosed lung cancer. CASE REPORT A 42-year-old female with a benign medical history presented with acute onset of bilateral hearing loss. The duration of this hearing loss was 2 weeks on the left and 3 days on the right. She also reported mild dizziness, headache, and vague visual changes. Cranial nerve examination was normal except for an obvious hearing loss; there was no nystagmus, facial nerve dysfunction, or other abnormality identified. Pure tone audiometry revealed a moderate-toprofound bilateral sensorineural hearing loss (Fig 1). Impedance testing was normal in both ears, and acoustic reflexes were present on the left but absent on the right. A postgadolinium T1-weighted MRI of the brain revealed enhancement of both vestibulocochlear nerves at the cerebellopontine angle (Fig 2). In addition, multiple contrastenhancing nodules were identified in the left cerebellum and the left frontal and occipital lobes. A chest x-ray showed a 4 × 5-cm mass in the right upper lobe and mediastinal lymphadenopathy. A percutaneous biopsy of the
Department of Otolaryngology–Head and Neck Surgery, University of Nebraska Medical Center. Reprint requests: Mary C. Snyder, MD, Department of Otolaryngology–Head and Neck Surgery, University of Nebraska Medical Center, 981225 Nebraska Medical Center, Omaha, NE 681981225; e-mail, [email protected]. Otolaryngol Head Neck Surg 2001;124:592-4. Copyright © 2001 by the American Academy of Otolaryngology– Head and Neck Surgery Foundation, Inc. 0194-5998/2001/$35.00 + 0 23/4/115057 doi:10.1067/mhn.2001.115057 592
lung lesion returned bronchogenic non–small cell carcinoma. Although a lumbar puncture was not performed, the patient was diagnosed with leptomeningeal carcinomatosis based on physical examination and MRI findings. Her neurologic symptoms were initially treated with steroids without improvement in hearing, dizziness, or headaches. She then underwent a course of whole-brain irradiation with concurrent chemotherapy (Taxol). A repeat MRI 2 months later showed persistent leptomeningeal disease. She is currently undergoing systemic chemotherapy at another institution for treatment of the primary tumor. DISCUSSION
Leptomeningeal carcinomatosis, or carcinomatous meningitis, is the diffuse seeding of the leptomeninges by systemic malignancy. This condition affects approximately 5% of all cancer patients, most commonly those with breast cancer, lung cancer, or melanoma.1 The majority of patients diagnosed with carcinomatous meningitis have a prior diagnosis of cancer. Leptomeningeal involvement as a presenting symptom of malignancy is rare.2 Most patients with carcinomatous meningitis have widely metastatic disease. Malignant cells reach the subarachnoid space by either hematogenous spread or direct extension from metastatic foci in the brain. After gaining access to the leptomeninges, neoplastic cells are circulated through the central nervous system by the cerebrospinal fluid (CSF).1 Patients with leptomeningeal carcinomatosis present with multifocal neurologic signs and symptoms indicative of cerebral, cranial nerve, and spinal root involvement. Headache is the most common cerebral symptom, followed by mental status changes, lightheadedness, nausea, ataxic gait, and seizures. Cranial nerve involvement may manifest with facial paralysis, diplopia, decreased visual acuity, dysphagia, dysarthria, hearing loss, tinnitus, and vertigo. Spinal root involvement manifests as back and radicular pain, weakness, and paresthesias.2 The diagnosis of leptomeningeal carcinomatosis is made on the basis of history, physical examination, imaging studies, and lumbar puncture with examination of CSF. Multiple subarachnoid mass lesions and abnormal leptomeningeal enhancement on CT or MRI is highly suggestive of and may be diagnostic of lep-
Otolaryngology– Head and Neck Surgery Volume 124 Number 5
SNYDER and HEYWOOD
593
Fig 1. Pure tone audiogram at presentation.
tomeningeal carcinomatosis in a patient with a known malignancy displaying multifocal neurologic signs and symptoms. Cytologic examination of the CSF is the gold standard for the diagnosis of meningeal carcinomatosis, although 50% of initial lumbar punctures are negative for malignant cells.1 Hearing loss associated with a metastatic malignancy is not a new phenomenon. In 1900, Saenger3 reported a case of deafness caused by subarachnoid invasion of the acoustic nerve by malignant cells from a breast adenocarcinoma. In 1965, Oshiro and Perlman4 reviewed 11 cases of hearing loss associated with carcinomatous meningitis. A postmortem examination of the temporal bone of 1 patient was conducted and revealed a tumor filling the internal auditory canal, destruction of the vestibulocochlear nerve, and infiltration of the facial nerve and geniculate ganglion by cancer cells. A recent report detailed similar findings on histopathologic examination of the temporal bone of a patient who presented with bilateral sensorineural hearing loss and facial paralysis caused by leptomeningeal carcinomatosis from a transitional cell carcinoma of the renal pelvis.5 This case represents an unusual presentation of a lung carcinoma. Review of the literature reveals only 2 reported cases of previously undiagnosed malignancy presenting as acute bilateral hearing loss; one a transitional cell carcinoma of the renal pelvis, the other a squamous cell carcinoma of the esophagus. 5,6 The acute onset of sensorineural hearing loss, particularly
Fig 2. Gadolinium-enhanced T1-weighted magnetic resonance image reveals abnormal enhancement of the vestibulocochlear nerves at the cerebellopontine angle (arrows).
if bilateral, should prompt a search for other cranial nerve deficits and consideration of leptomeningeal carcinomatosis, especially in a patient with a history of cancer.
594
SNYDER and HEYWOOD
REFERENCES 1. Grossman SA, Krabak MJ. Leptomeningeal carcinomatosis. Cancer Treat Rev 1999;25:103-19. 2. Olson ME, Chernik NL, Posner JB. Infiltration of the leptomeninges by systemic cancer: a clinical and pathological study. Arch Neurol 1974;30:122-37. 3. Saenger A. Über Hirnsymptomoe bei Carcinomatose. Muenchen Med Wschr 1900;47:341-2.
Otolaryngology– Head and Neck Surgery May 2001
4. Oshiro H, Perlman HB. Subarachnoid spread of tumor to the labyrinth. Arch Otolaryngol 1965;81:328-34. 5. Imamura S, Nozawa I, Imamura M, Murakami Y. Clinicopathologic study of leptomeningeal carcinomatosis involving the temporal bone. Ann Otol Rhinol Laryngol 1997;106:674-9. 6. Civantos F, Choi YS, Apllebaum EL. Meningeal carcinomatosis producing bilateral sudden hearing loss: a case report. Am J Otol 1992;13:369-71.
L
eptomeningeal carcinomatosis is the multifocal spread of tumor cells to the leptomeninges from a distant solid tumor, most commonly adenocarcinoma of the breast or lung, or melanoma. The overwhelming majority of cases are diagnosed in patients with a known malignancy. Affected patients present with acute, multifocal, neurologic deficits. Vestibulocochlear nerve involvement often results in sensorineural hearing loss, tinnitus, and vertigo. Although considered a relatively rare disorder, leptomeningeal carcinomatosis is being diagnosed more frequently as improved treatments provide cancer patients with longer survival times.1 We present a case of acute hearing loss as a result of leptomeningeal carcinomatosis in a patient with a previously undiagnosed lung cancer. CASE REPORT A 42-year-old female with a benign medical history presented with acute onset of bilateral hearing loss. The duration of this hearing loss was 2 weeks on the left and 3 days on the right. She also reported mild dizziness, headache, and vague visual changes. Cranial nerve examination was normal except for an obvious hearing loss; there was no nystagmus, facial nerve dysfunction, or other abnormality identified. Pure tone audiometry revealed a moderate-toprofound bilateral sensorineural hearing loss (Fig 1). Impedance testing was normal in both ears, and acoustic reflexes were present on the left but absent on the right. A postgadolinium T1-weighted MRI of the brain revealed enhancement of both vestibulocochlear nerves at the cerebellopontine angle (Fig 2). In addition, multiple contrastenhancing nodules were identified in the left cerebellum and the left frontal and occipital lobes. A chest x-ray showed a 4 × 5-cm mass in the right upper lobe and mediastinal lymphadenopathy. A percutaneous biopsy of the
Department of Otolaryngology–Head and Neck Surgery, University of Nebraska Medical Center. Reprint requests: Mary C. Snyder, MD, Department of Otolaryngology–Head and Neck Surgery, University of Nebraska Medical Center, 981225 Nebraska Medical Center, Omaha, NE 681981225; e-mail, [email protected]. Otolaryngol Head Neck Surg 2001;124:592-4. Copyright © 2001 by the American Academy of Otolaryngology– Head and Neck Surgery Foundation, Inc. 0194-5998/2001/$35.00 + 0 23/4/115057 doi:10.1067/mhn.2001.115057 592
lung lesion returned bronchogenic non–small cell carcinoma. Although a lumbar puncture was not performed, the patient was diagnosed with leptomeningeal carcinomatosis based on physical examination and MRI findings. Her neurologic symptoms were initially treated with steroids without improvement in hearing, dizziness, or headaches. She then underwent a course of whole-brain irradiation with concurrent chemotherapy (Taxol). A repeat MRI 2 months later showed persistent leptomeningeal disease. She is currently undergoing systemic chemotherapy at another institution for treatment of the primary tumor. DISCUSSION
Leptomeningeal carcinomatosis, or carcinomatous meningitis, is the diffuse seeding of the leptomeninges by systemic malignancy. This condition affects approximately 5% of all cancer patients, most commonly those with breast cancer, lung cancer, or melanoma.1 The majority of patients diagnosed with carcinomatous meningitis have a prior diagnosis of cancer. Leptomeningeal involvement as a presenting symptom of malignancy is rare.2 Most patients with carcinomatous meningitis have widely metastatic disease. Malignant cells reach the subarachnoid space by either hematogenous spread or direct extension from metastatic foci in the brain. After gaining access to the leptomeninges, neoplastic cells are circulated through the central nervous system by the cerebrospinal fluid (CSF).1 Patients with leptomeningeal carcinomatosis present with multifocal neurologic signs and symptoms indicative of cerebral, cranial nerve, and spinal root involvement. Headache is the most common cerebral symptom, followed by mental status changes, lightheadedness, nausea, ataxic gait, and seizures. Cranial nerve involvement may manifest with facial paralysis, diplopia, decreased visual acuity, dysphagia, dysarthria, hearing loss, tinnitus, and vertigo. Spinal root involvement manifests as back and radicular pain, weakness, and paresthesias.2 The diagnosis of leptomeningeal carcinomatosis is made on the basis of history, physical examination, imaging studies, and lumbar puncture with examination of CSF. Multiple subarachnoid mass lesions and abnormal leptomeningeal enhancement on CT or MRI is highly suggestive of and may be diagnostic of lep-
Otolaryngology– Head and Neck Surgery Volume 124 Number 5
SNYDER and HEYWOOD
593
Fig 1. Pure tone audiogram at presentation.
tomeningeal carcinomatosis in a patient with a known malignancy displaying multifocal neurologic signs and symptoms. Cytologic examination of the CSF is the gold standard for the diagnosis of meningeal carcinomatosis, although 50% of initial lumbar punctures are negative for malignant cells.1 Hearing loss associated with a metastatic malignancy is not a new phenomenon. In 1900, Saenger3 reported a case of deafness caused by subarachnoid invasion of the acoustic nerve by malignant cells from a breast adenocarcinoma. In 1965, Oshiro and Perlman4 reviewed 11 cases of hearing loss associated with carcinomatous meningitis. A postmortem examination of the temporal bone of 1 patient was conducted and revealed a tumor filling the internal auditory canal, destruction of the vestibulocochlear nerve, and infiltration of the facial nerve and geniculate ganglion by cancer cells. A recent report detailed similar findings on histopathologic examination of the temporal bone of a patient who presented with bilateral sensorineural hearing loss and facial paralysis caused by leptomeningeal carcinomatosis from a transitional cell carcinoma of the renal pelvis.5 This case represents an unusual presentation of a lung carcinoma. Review of the literature reveals only 2 reported cases of previously undiagnosed malignancy presenting as acute bilateral hearing loss; one a transitional cell carcinoma of the renal pelvis, the other a squamous cell carcinoma of the esophagus. 5,6 The acute onset of sensorineural hearing loss, particularly
Fig 2. Gadolinium-enhanced T1-weighted magnetic resonance image reveals abnormal enhancement of the vestibulocochlear nerves at the cerebellopontine angle (arrows).
if bilateral, should prompt a search for other cranial nerve deficits and consideration of leptomeningeal carcinomatosis, especially in a patient with a history of cancer.
594
SNYDER and HEYWOOD
REFERENCES 1. Grossman SA, Krabak MJ. Leptomeningeal carcinomatosis. Cancer Treat Rev 1999;25:103-19. 2. Olson ME, Chernik NL, Posner JB. Infiltration of the leptomeninges by systemic cancer: a clinical and pathological study. Arch Neurol 1974;30:122-37. 3. Saenger A. Über Hirnsymptomoe bei Carcinomatose. Muenchen Med Wschr 1900;47:341-2.
Otolaryngology– Head and Neck Surgery May 2001
4. Oshiro H, Perlman HB. Subarachnoid spread of tumor to the labyrinth. Arch Otolaryngol 1965;81:328-34. 5. Imamura S, Nozawa I, Imamura M, Murakami Y. Clinicopathologic study of leptomeningeal carcinomatosis involving the temporal bone. Ann Otol Rhinol Laryngol 1997;106:674-9. 6. Civantos F, Choi YS, Apllebaum EL. Meningeal carcinomatosis producing bilateral sudden hearing loss: a case report. Am J Otol 1992;13:369-71.