- Email: [email protected]
Leptomeningeal carcinomatosis: An unusual cause of sudden onset bilateral sensorineural hearing loss
116
Case reports / Journal of Clinical Neuroscience 13 (2006) 116–118
Fig. 2. Patient 2. T1-weighted contrast enhanced MRI. The meningioma was located on the inferior parietal cortex. AG, angular gyrus; CS, central sulcus; IPS, intraparietal sulcus; Rt., right; SMG, supramarginal gyrus; SPG, superior parietal gyrus; STG, superior temporal gyrus; STS, superior temporal sulcus; PCS, post central sulcus.
with the hypothesis that the human inferior parietal cortex may be functionally homologous to monkey area 7. Although we have discussed these human vestibular cortices based on MRI findings, there remains the possibility that the lesions exerted a remote effect. Information on similar cases, with detailed neurophysiological evaluations, including electrocorticography, are required. References 1. Brandt T, Dieterich M. The vestibular cortex. Its locations, functions, and disorders. Ann NY Acad Sci 1999;871:293–312.
2. Odkvist L, Schwartz D, Fredrickson J, Hassler R. Projection of the vestibular nerve to the area 3a arm field in the squirrel monkey (Saimiri sciureus). Exp Brain Res 1974;21:97–105. 3. Yousry T, Schmid U, Alkadhi H, et al. Localization of the motor hand area to a knob on the precentral gyrus. A new landmark. Brain 1997;120:141–57. 4. Luders HO, Awad I. Conceptual considerations. In: Luders HO, editor. Epilepsy Surgery. New York: Raven Press; 1992. p. 51–62. 5. Brandt T, Dieterich M, Danek A. Vestibular cortex lesions affect the perception of verticality. Ann Neurol 1994;35:403–12. 6. Andersen RA, Gnadt JW. Posterior parietal cortex. In: Wurtz RH, Goldberg ME, editors. Reviews of Oculomotor Research. The Neurobiology of Saccadic Eye Movements, vol. 3. Amsterdam: Elsevier; 1989. p. 315–35.
doi:10.1016/j.jocn.2004.11.022
Leptomeningeal carcinomatosis: An unusual cause of sudden onset bilateral sensorineural hearing loss Graham Joseph Jeffs *, Gabriel Yin-Foo Lee, George Tse-Hwai Wong Department of Neurosurgery, Sir Charles Gairdiner Hospital, Perth 6008, Western Australia, Australia Received 13 February 2004; accepted 20 December 2004
Abstract We report a 66-year-old woman who developed sudden-onset bilateral sensorineural deafness due to leptomeningeal carcinomatosis involving the vestibulocochlear nerves. The clinical and diagnostic features of leptomeningeal carcinomatosis are discussed. 2005 Elsevier Ltd. All rights reserved. *
Corresponding author. Tel.: +61 0 8 9346 2865; fax: +61 0 8 93463824. E-mail address: jeff[email protected] (G.J. Jeffs).
Case reports / Journal of Clinical Neuroscience 13 (2006) 116–118
117
Keywords: Leptomeningeal carcinomatosis; Cranial nerves; Melanoma; Sensorineural hearing loss
1. Introduction Leptomeningeal carcinomatosis is defined as diffuse involvement of the leptomeninges by metastatic cancer. The entire neuraxis can be affected, causing an array of neurological signs and symptoms.1,2 We present an unusual case of sudden bilateral sensorineural hearing loss caused by infiltration of the leptomeninges with metastatic melanoma. Improvements in imaging for leptomeningeal disease may obviate the requirement for cerebrospinal fluid (CSF) sampling in selected cases. 2. Case report A 66-year-old woman with a history of metastatic melanoma presented with sudden onset headache and visual disturbance. Neurological testing revealed a left homonymous hemianopia, and a CT scan revealed a 3 cm · 3 cm haemorrhage in the right parietal lobe. Underlying metastasis was strongly suspected, but MRI failed to reveal an obvious lesion. The patient was treated conservatively and discharged from hospital. A follow-up MRI scan 6 weeks after discharge, however, revealed a mass in the right parietal lobe. The decision was made to resect the lesion and the patient was readmitted. Preoperatively, the patient had no new symptoms, and neurological testing was normal except for the persisting left homonymous hemianopia. Resection of the parietal tumor was performed via a right parietal craniotomy. Histopathological tests confirmed the diagnosis of metastatic melanoma. The patient made a good initial recovery until 7 days after the operation when she complained of suddenonset bilateral hearing loss. She had no other new symptoms, and cranial nerve testing revealed no other abnormalities. There was no vertigo or nystagmus. Caloric testing was not performed. Examination of the external auditory meati did not reveal any abnormalities, and a review of the notes did not reveal any neurotoxic or ototoxic medications. An audiogram indicated severe bilateral sensorineural deafness. An MRI scan was performed to ascertain the cause of the hearing loss. Numerous intraventricular epen-
dymal nodules were seen, and there was bilateral thickening and enhancement with gadolinium of both the vestibulo-cochlear nerves within the internal acoustic meati, consistent with leptomeningeal carcinomatosis (Fig. 1). The patientÕs steroid dose was increased and she was referred for urgent adjuvant radiotherapy. Her hearing loss did not improve, however, and her condition deteriorated. The patient died 2 weeks after the onset of hearing loss. 3. Discussion Differential diagnosis for bilateral sensorineural hearing loss includes presbyacusis, ototoxic drugs such as aminoglycosides, infection, environmental noise exposure, bilateral acoustic neuroma and MeniereÕs disease. However, sudden-onset bilateral sensorineural hearing loss is rare in these conditions. We present here the unusual case of a woman with metastatic melanoma, who experienced sudden bilateral hearing loss due to infiltration of the vestibulocochlear nerves by tumour. Leptomeningeal carcinomatosis can affect any level of the neuraxis, causing signs and symptoms through disturbance of the cranial nerves, spinal cord or cerebral hemispheres. Most patients have a known primary tumor, but in some cases, leptomeningeal involvement is the first sign of cancer.1,2 The most common solid tumours causing leptomeningeal carcinomatosis are adenocarcinomas of the breast and lung, followed by cutaneous melanoma.2,3 The primary tumor can spread to the meninges in a number of ways. Haematogenous spread via the choroid plexus or meningeal vessels, perineural invasion and proximal extension, lymphatic invasion or spread from primary cerebrospinal tumours are all possible routes.1,4 Cranial nerve involvement is usually multiple and the occulomotor (III), trigeminal (V) and facial (VII) nerves are the most commonly affected.5 Although vestibulo-cochlear nerve (VIII) involvement has been reported in up to 10% of patients with leptomeningeal carcinomatosis at autopsy,4 presentation with isolated hearing loss is very rare. Saenger presented the first case in 1900,6 and since then there have
Fig. 1. Gadolinium-enhanced T1-weighted MRI images demonstrating abnormal thickening and enhancement of the vestibulocochlear nerves within the internal acoustic meati bilaterally (arrows).
118
Case reports / Journal of Clinical Neuroscience 13 (2006) 118–121
been only a few added cases of isolated bilateral sudden-onset deafness as a result of leptomeningeal malignancy. Alberts and Terrence reported five cases of leptomeningeal carcinomatosis with vestibulo-cochlear nerve involvement.4 All patients presented initially with unilateral deafness and tinnitus, which rapidly progressed to bilateral hearing loss. Several reports have described sudden-onset hearing loss as the first presentation of cancer.1,5,7 The diagnosis of leptomeningeal carcinomatosis can be difficult to make. Positive CSF cytology is the historical gold standard, but several authors have stressed the need for multiple lumbar punctures to yield neoplastic cells.1–3,5,8 Freilich et al., in a review of CSF and neuroimaging findings, suggested that contrast-enhanced MRI scanning with typical clinical features is adequate for a diagnosis of leptomeningeal carcinomatosis.9 This holds even when CSF cytology is negative. Von Campe et al. reported a 70% sensitivity for MRI in the detection of leptomeningeal carcinomatosis proven at autopsy.10 The exact mechanism underlying the sudden hearing loss is unclear, but leptomeningeal spread within the confines of the internal auditory meatus may cause sudden-onset hearing loss by direct compression within rigid surrounds. Vascular compromise may occur, as well as direct invasion and axonal destruction.5,11 The prognosis in cases of leptomeningeal carcinomatosis is generally dismal, with a median survival of 6 months in treated patients.12 Treatment options include intrathecal chemotherapy and whole-brain radiotherapy. Steroids are often employed to reduce oedema.1–3,12 Recovery or improvement of the hearing loss with treatment has not been reported. In conclusion, leptomeningeal carcinomatosis should be considered in the differential diagnosis in cases of abruptonset sensorineural hearing loss. This applies even when there is no known primary malignancy. MRI scanning is
an important investigation tool, because it is sensitive for leptomeningeal carcinomatosis, and may also reveal other causes of sensorineural hearing loss, such as acoustic neuromas. MRI scans suggestive of leptomeningeal carcinomatosis combined with typical clinical features are strongly suggestive of leptomeningeal carcinomatosis, and we suggest that CSF sampling may be unwarranted in these cases, given the reported difficulty in obtaining a positive result. References 1. Uppal HS, Ayshford CA, Wilson F. Sudden onset bilateral sensorineural hearing loss: A manifestation of occult breast carcinoma. J Laryngol Otol 2001;115:907–11. 2. Morgan MK, Zammit-Maempel I, Hill J. Meningeal carcinomatosis: An unusual cause of deafness. J R Coll Edinb 1998;43:119–21. 3. Siegal G, Braun J, Kuten A, et al. Sensorineural hearing loss and metastatic leptomeningeal malignancy. Isr Med Assoc J 2002;4:300–1. 4. Alberts MC, Terrence CF. Hearing loss in carcinomatous meningitis. J Laryngol Otol 1978;92:233–41. 5. Civantos F, Choi YS, Applebaum EL. Meningeal carcinomatosis producing sudden hearing loss: A case report. Am J Otol 1992;13:369–71. 6. Saenger A. Uber hirnsymptome bei carcinomatose. Munch Med Wochenschr 1900;38:51–7. 7. Imamura S, Nozawa I, Imamura M, Murakawi Y. Clinicopathological study of leptomeningeal carcinomatosis involving the temporal bone. Ann Otol Rhinol Laryngol 1997;106:674–9. 8. Shen TY, Young YH. Meningeal carcinomatosis manifested as bilateral progressive sensorineural hearing loss. Am J Otol 2000;21:510–2. 9. Freilich RJ, Krol G, DeAngelis LM. Neuroimaging and cerebrospinal fluid cytology in the diagnosis of leptomeningeal metastasis. Ann Neurol 1995;38:51–7. 10. Von Campe G, Regli F, Confavreux C. Surdite bilaterale, manifestation initiale dÕune carcinomatose meningee. Rev Neurol 1992;148: 133–8. 11. Oshiro H, Perlman HB. Subarachnoid spread of tumours in the labyrinth. Arch Otolaryngol Head Neck Surg 1965;81:328–34. 12. Chamberlain MC. New approaches to and current treatment of leptomeningeal metastasis. Curr Opin Neurol 1994;7:492–500.
doi:10.1016/j.jocn.2004.12.009
Spontaneous cerebrospinal fluid rhinorrhea following excision of a massive torcular meningioma Trimurti D. Nadkarni *, Ram Kumar Menon, Ketan I. Desai, Atul Goel Department of Neurosurgery, King Edward Memorial Hospital, Seth G.S. Medical College, Parel, Mumbai 400 012, India Received 17 August 2004; accepted 14 January 2005
Abstract A massive supratentorial torcular meningioma was completely resected in a 50-year-old woman. On the tenth postoperative day she presented with spontaneous cerebrospinal fluid (CSF) rhinorrhea. The site of the CSF leak was through the region of the crib*
Corresponding author. Tel.: +91 22 24129884; fax: +91 22 24143435. E-mail address: [email protected] (T.D. Nadkarni).
Case reports / Journal of Clinical Neuroscience 13 (2006) 116–118
Fig. 2. Patient 2. T1-weighted contrast enhanced MRI. The meningioma was located on the inferior parietal cortex. AG, angular gyrus; CS, central sulcus; IPS, intraparietal sulcus; Rt., right; SMG, supramarginal gyrus; SPG, superior parietal gyrus; STG, superior temporal gyrus; STS, superior temporal sulcus; PCS, post central sulcus.
with the hypothesis that the human inferior parietal cortex may be functionally homologous to monkey area 7. Although we have discussed these human vestibular cortices based on MRI findings, there remains the possibility that the lesions exerted a remote effect. Information on similar cases, with detailed neurophysiological evaluations, including electrocorticography, are required. References 1. Brandt T, Dieterich M. The vestibular cortex. Its locations, functions, and disorders. Ann NY Acad Sci 1999;871:293–312.
2. Odkvist L, Schwartz D, Fredrickson J, Hassler R. Projection of the vestibular nerve to the area 3a arm field in the squirrel monkey (Saimiri sciureus). Exp Brain Res 1974;21:97–105. 3. Yousry T, Schmid U, Alkadhi H, et al. Localization of the motor hand area to a knob on the precentral gyrus. A new landmark. Brain 1997;120:141–57. 4. Luders HO, Awad I. Conceptual considerations. In: Luders HO, editor. Epilepsy Surgery. New York: Raven Press; 1992. p. 51–62. 5. Brandt T, Dieterich M, Danek A. Vestibular cortex lesions affect the perception of verticality. Ann Neurol 1994;35:403–12. 6. Andersen RA, Gnadt JW. Posterior parietal cortex. In: Wurtz RH, Goldberg ME, editors. Reviews of Oculomotor Research. The Neurobiology of Saccadic Eye Movements, vol. 3. Amsterdam: Elsevier; 1989. p. 315–35.
doi:10.1016/j.jocn.2004.11.022
Leptomeningeal carcinomatosis: An unusual cause of sudden onset bilateral sensorineural hearing loss Graham Joseph Jeffs *, Gabriel Yin-Foo Lee, George Tse-Hwai Wong Department of Neurosurgery, Sir Charles Gairdiner Hospital, Perth 6008, Western Australia, Australia Received 13 February 2004; accepted 20 December 2004
Abstract We report a 66-year-old woman who developed sudden-onset bilateral sensorineural deafness due to leptomeningeal carcinomatosis involving the vestibulocochlear nerves. The clinical and diagnostic features of leptomeningeal carcinomatosis are discussed. 2005 Elsevier Ltd. All rights reserved. *
Corresponding author. Tel.: +61 0 8 9346 2865; fax: +61 0 8 93463824. E-mail address: jeff[email protected] (G.J. Jeffs).
Case reports / Journal of Clinical Neuroscience 13 (2006) 116–118
117
Keywords: Leptomeningeal carcinomatosis; Cranial nerves; Melanoma; Sensorineural hearing loss
1. Introduction Leptomeningeal carcinomatosis is defined as diffuse involvement of the leptomeninges by metastatic cancer. The entire neuraxis can be affected, causing an array of neurological signs and symptoms.1,2 We present an unusual case of sudden bilateral sensorineural hearing loss caused by infiltration of the leptomeninges with metastatic melanoma. Improvements in imaging for leptomeningeal disease may obviate the requirement for cerebrospinal fluid (CSF) sampling in selected cases. 2. Case report A 66-year-old woman with a history of metastatic melanoma presented with sudden onset headache and visual disturbance. Neurological testing revealed a left homonymous hemianopia, and a CT scan revealed a 3 cm · 3 cm haemorrhage in the right parietal lobe. Underlying metastasis was strongly suspected, but MRI failed to reveal an obvious lesion. The patient was treated conservatively and discharged from hospital. A follow-up MRI scan 6 weeks after discharge, however, revealed a mass in the right parietal lobe. The decision was made to resect the lesion and the patient was readmitted. Preoperatively, the patient had no new symptoms, and neurological testing was normal except for the persisting left homonymous hemianopia. Resection of the parietal tumor was performed via a right parietal craniotomy. Histopathological tests confirmed the diagnosis of metastatic melanoma. The patient made a good initial recovery until 7 days after the operation when she complained of suddenonset bilateral hearing loss. She had no other new symptoms, and cranial nerve testing revealed no other abnormalities. There was no vertigo or nystagmus. Caloric testing was not performed. Examination of the external auditory meati did not reveal any abnormalities, and a review of the notes did not reveal any neurotoxic or ototoxic medications. An audiogram indicated severe bilateral sensorineural deafness. An MRI scan was performed to ascertain the cause of the hearing loss. Numerous intraventricular epen-
dymal nodules were seen, and there was bilateral thickening and enhancement with gadolinium of both the vestibulo-cochlear nerves within the internal acoustic meati, consistent with leptomeningeal carcinomatosis (Fig. 1). The patientÕs steroid dose was increased and she was referred for urgent adjuvant radiotherapy. Her hearing loss did not improve, however, and her condition deteriorated. The patient died 2 weeks after the onset of hearing loss. 3. Discussion Differential diagnosis for bilateral sensorineural hearing loss includes presbyacusis, ototoxic drugs such as aminoglycosides, infection, environmental noise exposure, bilateral acoustic neuroma and MeniereÕs disease. However, sudden-onset bilateral sensorineural hearing loss is rare in these conditions. We present here the unusual case of a woman with metastatic melanoma, who experienced sudden bilateral hearing loss due to infiltration of the vestibulocochlear nerves by tumour. Leptomeningeal carcinomatosis can affect any level of the neuraxis, causing signs and symptoms through disturbance of the cranial nerves, spinal cord or cerebral hemispheres. Most patients have a known primary tumor, but in some cases, leptomeningeal involvement is the first sign of cancer.1,2 The most common solid tumours causing leptomeningeal carcinomatosis are adenocarcinomas of the breast and lung, followed by cutaneous melanoma.2,3 The primary tumor can spread to the meninges in a number of ways. Haematogenous spread via the choroid plexus or meningeal vessels, perineural invasion and proximal extension, lymphatic invasion or spread from primary cerebrospinal tumours are all possible routes.1,4 Cranial nerve involvement is usually multiple and the occulomotor (III), trigeminal (V) and facial (VII) nerves are the most commonly affected.5 Although vestibulo-cochlear nerve (VIII) involvement has been reported in up to 10% of patients with leptomeningeal carcinomatosis at autopsy,4 presentation with isolated hearing loss is very rare. Saenger presented the first case in 1900,6 and since then there have
Fig. 1. Gadolinium-enhanced T1-weighted MRI images demonstrating abnormal thickening and enhancement of the vestibulocochlear nerves within the internal acoustic meati bilaterally (arrows).
118
Case reports / Journal of Clinical Neuroscience 13 (2006) 118–121
been only a few added cases of isolated bilateral sudden-onset deafness as a result of leptomeningeal malignancy. Alberts and Terrence reported five cases of leptomeningeal carcinomatosis with vestibulo-cochlear nerve involvement.4 All patients presented initially with unilateral deafness and tinnitus, which rapidly progressed to bilateral hearing loss. Several reports have described sudden-onset hearing loss as the first presentation of cancer.1,5,7 The diagnosis of leptomeningeal carcinomatosis can be difficult to make. Positive CSF cytology is the historical gold standard, but several authors have stressed the need for multiple lumbar punctures to yield neoplastic cells.1–3,5,8 Freilich et al., in a review of CSF and neuroimaging findings, suggested that contrast-enhanced MRI scanning with typical clinical features is adequate for a diagnosis of leptomeningeal carcinomatosis.9 This holds even when CSF cytology is negative. Von Campe et al. reported a 70% sensitivity for MRI in the detection of leptomeningeal carcinomatosis proven at autopsy.10 The exact mechanism underlying the sudden hearing loss is unclear, but leptomeningeal spread within the confines of the internal auditory meatus may cause sudden-onset hearing loss by direct compression within rigid surrounds. Vascular compromise may occur, as well as direct invasion and axonal destruction.5,11 The prognosis in cases of leptomeningeal carcinomatosis is generally dismal, with a median survival of 6 months in treated patients.12 Treatment options include intrathecal chemotherapy and whole-brain radiotherapy. Steroids are often employed to reduce oedema.1–3,12 Recovery or improvement of the hearing loss with treatment has not been reported. In conclusion, leptomeningeal carcinomatosis should be considered in the differential diagnosis in cases of abruptonset sensorineural hearing loss. This applies even when there is no known primary malignancy. MRI scanning is
an important investigation tool, because it is sensitive for leptomeningeal carcinomatosis, and may also reveal other causes of sensorineural hearing loss, such as acoustic neuromas. MRI scans suggestive of leptomeningeal carcinomatosis combined with typical clinical features are strongly suggestive of leptomeningeal carcinomatosis, and we suggest that CSF sampling may be unwarranted in these cases, given the reported difficulty in obtaining a positive result. References 1. Uppal HS, Ayshford CA, Wilson F. Sudden onset bilateral sensorineural hearing loss: A manifestation of occult breast carcinoma. J Laryngol Otol 2001;115:907–11. 2. Morgan MK, Zammit-Maempel I, Hill J. Meningeal carcinomatosis: An unusual cause of deafness. J R Coll Edinb 1998;43:119–21. 3. Siegal G, Braun J, Kuten A, et al. Sensorineural hearing loss and metastatic leptomeningeal malignancy. Isr Med Assoc J 2002;4:300–1. 4. Alberts MC, Terrence CF. Hearing loss in carcinomatous meningitis. J Laryngol Otol 1978;92:233–41. 5. Civantos F, Choi YS, Applebaum EL. Meningeal carcinomatosis producing sudden hearing loss: A case report. Am J Otol 1992;13:369–71. 6. Saenger A. Uber hirnsymptome bei carcinomatose. Munch Med Wochenschr 1900;38:51–7. 7. Imamura S, Nozawa I, Imamura M, Murakawi Y. Clinicopathological study of leptomeningeal carcinomatosis involving the temporal bone. Ann Otol Rhinol Laryngol 1997;106:674–9. 8. Shen TY, Young YH. Meningeal carcinomatosis manifested as bilateral progressive sensorineural hearing loss. Am J Otol 2000;21:510–2. 9. Freilich RJ, Krol G, DeAngelis LM. Neuroimaging and cerebrospinal fluid cytology in the diagnosis of leptomeningeal metastasis. Ann Neurol 1995;38:51–7. 10. Von Campe G, Regli F, Confavreux C. Surdite bilaterale, manifestation initiale dÕune carcinomatose meningee. Rev Neurol 1992;148: 133–8. 11. Oshiro H, Perlman HB. Subarachnoid spread of tumours in the labyrinth. Arch Otolaryngol Head Neck Surg 1965;81:328–34. 12. Chamberlain MC. New approaches to and current treatment of leptomeningeal metastasis. Curr Opin Neurol 1994;7:492–500.
doi:10.1016/j.jocn.2004.12.009
Spontaneous cerebrospinal fluid rhinorrhea following excision of a massive torcular meningioma Trimurti D. Nadkarni *, Ram Kumar Menon, Ketan I. Desai, Atul Goel Department of Neurosurgery, King Edward Memorial Hospital, Seth G.S. Medical College, Parel, Mumbai 400 012, India Received 17 August 2004; accepted 14 January 2005
Abstract A massive supratentorial torcular meningioma was completely resected in a 50-year-old woman. On the tenth postoperative day she presented with spontaneous cerebrospinal fluid (CSF) rhinorrhea. The site of the CSF leak was through the region of the crib*
Corresponding author. Tel.: +91 22 24129884; fax: +91 22 24143435. E-mail address: [email protected] (T.D. Nadkarni).