Otolaryngology Case Reports 2 (2017) 7e9
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Sensorineural hearing loss and acute vestibulopathy in HELLP syndrome: A case report Edwin Halliday*, Neil Tan Department of Otolaryngology, Box 48, Addenbrooke's Hospital, Hills Road, Cambridge, CB2 0QQ, United Kingdom
a r t i c l e i n f o
a b s t r a c t
Article history: Received 11 October 2016 Accepted 24 January 2017 Available online 27 January 2017
HELLP syndrome is a complication of pregnancy characterised by haemolysis (H), elevated liver enzymes (EL) and low platelets (LP). Patients present with non-specific symptoms; delivery of the baby is the only treatment. We present the case of a woman who developed sudden sensorineural hearing loss during her HELLP syndrome episode. Poorly perfused placental tissue may release factors into the systemic circulation causing endothelial cell injury and a dysfunctional cascade of coagulation, vasoconstriction and intravascular fluid redistribution. There may be subsequent damage to the endothelium of the vasa nervorum of the vestibulocochlear nerve, resulting in sensorineural hearing loss and acute vestibulopathy. © 2017 Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/ licenses/by/4.0/).
Keywords: Hearing loss Sudden sensorineural hearing loss Acute vestibulopathy Pregnancy HELLP syndrome
1. Introduction HELLP syndrome is a life threatening complication of pregnancy that is characterised by haemolysis (H), elevated liver enzymes (EL) and low platelets (LP). It occurs in 0.2e0.6% of all pregnancies and in 10e20% of women with severe pre-eclampsia [1,2]. HELLP syndrome increases the risk of maternal and neonatal morbidity and mortality [1,2]. Risk factors include multiparity, maternal age, previous pregnancy with eclampsia or pre-eclampsia and family history of HELLP syndrome [3]. Patients present with non-specific symptoms which can include abdominal, thoracic or right shoulder pain. Delivery of the baby is the only effective treatment. The exact pathophysiology of the disease has not been clearly established. It is not clear if HELLP syndrome is a severe form of preeclampsia or a distinct clinical entity, although it is assumed that the syndrome results from abnormal placental development with altered placental function resulting in ischemia-producing oxidative stress [4]. The syndrome features thrombotic microangiopathy: consumptive thrombocytopenia with the formation of microvascular platelet thrombi, resulting in microangiopathic haemolytic anaemia and hepatic damage [5]. Pathogenesis may also involve
Abbreviations: CROS, contralateral routing of signals hearing aid; HELLP, haemolysis (H), elevated liver enzymes (EL) and low platelets (LP); TEOAE, transient evoked otoacoustic emission. * Corresponding author. E-mail address:
[email protected] (E. Halliday).
maternal vascular endothelial dysfunction with resultant impaired synthesis of vasodilators or excessive production of vasoconstrictors [6]. To the authors' knowledge, no previous case reports have been published describing sensorineural hearing loss in patients with HELLP syndrome. 2. Case presentation A 20 year old woman was transferred to a tertiary hospital under the care of the hepatology team with HELLP syndrome. She had previously presented three days earlier to the district hospital at 33 þ 3 weeks pregnant with severe right upper quadrant pain and a platelet count of 88 109/L. She underwent an emergency caesarean section the next day with minimal blood loss. The platelet count four hours after delivery was 135 109/L. The abdominal pain improved after surgery. Her past medical history includes previous termination of pregnancy at 23 weeks for severe pre-eclampsia and HELLP syndrome, and she had also suffered from a pulmonary embolism. She was known to be strongly positive for lupus anticoagulant, anticardiolipin and anti-beta 2 glycoprotein 1 antibodies. Her only regular medication was aspirin 75mg once daily. The day after the caesarean section the patient started to develop chest and abdominal pain. A CT pulmonary angiogram showed no pulmonary embolism but did identify multiple liver infarcts. Transfer was arranged to the tertiary hospital where she reported back and abdominal pain. On examination her abdomen
http://dx.doi.org/10.1016/j.xocr.2017.01.002 2468-5488/© 2017 Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
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was soft but there was generalised tenderness. Blood tests showed: anaemia (Hb 88 g/L), elevated liver enzymes (alanine transaminase 1819 U/L, alkaline phosphatase 171 U/L) and low platelets (37 109/L). The impression was one of ischaemic hepatitis on a background of HELLP syndrome and she was treated with analgesia, intravenous fluids, magnesium sulphate infusion, intravenous antibiotics, N-acetylcysteine and prophylactic doses of low molecular weight heparin. The day after transfer the patient was feeling much better and the blood tests appeared to have reached their peak. However, she now reported that while she had been admitted to the district hospital she noticed sudden onset left sided hearing loss associated with tinnitus and vertigo. This hearing loss had persisted. There was no otalgia or discharge and she had no previous otological history. On examination otoscopy was unremarkable and the seventh cranial nerve was intact. Weber's test lateralised to the right, while Rinne's test was negative on the left and positive on the right. An audiogram showed unilateral left sided profound sensorineural hearing loss (Fig. 1). She was treated with an intratympanic injection of steroids (0.8ml methylprednisolone; 40mg/ml), betahistine and aciclovir for two weeks. Three months later, the patient continued to report poor hearing from the left ear and tinnitus which was affecting her life negatively. She did not feel that the steroid injection had made any difference to her symptoms. An MRI scan of the internal auditory canals and posterior fossa was performed which was normal and a repeat audiogram showed no improvement in hearing thresholds (Fig. 2). She was referred to the audiology clinic for assessment and tinnitus management and agreed to try a relaxation CD, a digital environmental sound generator and a contralateral routing of signals (CROS) hearing aid. The patient also had a follow-up MRI scan of the liver three months after admission. This showed normal appearance of the liver with no areas of focal ischaemia seen. Repeat blood tests
Fig. 1. Pure tone audiogram performed within a few days of the onset of symptoms showing unilateral left sided profound sensorineural hearing loss.
Fig. 2. Pure tone audiogram performed at seven months showing no improvement in hearing thresholds.
showed that the haemoglobin level, platelet count and liver function tests had returned to normal. 3. Discussion We present the case of a woman who reported sudden hearing loss and vestibulopathy during the acute phase of her HELLP syndrome episode. One prospective, case-control study compared hearing loss between normal and hypertensive women during the first week after delivery, using transient evoked otoacoustic emission (TEOAE) testing [7]. The hypertensive group included women with gestational hypertension, pre-eclampsia, eclampsia and HELLP syndrome. The study noted that there was a significantly higher ratio of hearing loss with TEOAE in women with HELLP syndrome compared to those with severe and mild eclampsia. Other variants of hypertensive disorders in pregnancy were not found to impair hearing function. To our knowledge, no previous case reports have been published describing hearing loss in patients with HELLP. The cochlea is supplied by an end artery and vascular occlusion has been suggested as a cause for sudden sensorineural hearing loss as early as 1949 [8]. Studies have investigated several possible mechanisms including atherosclerosis, hypotension, thrombophilia, vasospasm, hyperviscosity and paradoxical embolism [9]. Hyperviscosity syndromes such as sickle-cell syndrome, Waldenstrom's macroglobulinaemia and myeloma may be associated with sudden sensorineural hearing loss. Vascular pathology may be the underlying mechanism for sudden sensorineural hearing loss in giant-cell arteritis, Takayasu's arteritis, Wegener's granulomatosis, € gren's syndrome, relapsing polychondritis systemic sclerosis, Sjo and polyarteritis nodosa. While the exact pathophysiology of HELLP syndrome has not been clearly established, and it is not clear if the syndrome is a severe form of pre-eclampsia or a distinct clinical entity, it is thought that the syndrome results from abnormal placental development with altered placental function resulting in ischemia-
E. Halliday, N. Tan / Otolaryngology Case Reports 2 (2017) 7e9
producing oxidative stress [4]. In pre-eclampsia, and potentially also HELLP syndrome, there appears to be endothelial cell injury which reduces the synthesis of vasorelaxing agents, increases the production of vasoconstrictors, impairs synthesis of endogenous anticoagulants and increases procoagulant production [6]. In pre-eclampsia, evidence of endothelial injury is illustrated by glomerular endotheliosis [10]. This condition is characterised by glomerular endothelial swelling with loss of endothelial fenestrae and occlusion of the capillary lumens. This glomerular lesion appears to be mediated by a soluble vascular endothelial growth factor receptor that deprives glomerular endothelial cells of the vascular endothelial growth factor that they require, leading to cellular injury and disruption of the filtration apparatus with subsequent proteinuria [10]. In HELLP syndrome, poorly perfused placental tissue is likely to release a number of factors into the systemic circulation that causes injury to endothelial cells. The endothelial cell injury may set in motion a dysfunctional cascade of coagulation, vasoconstriction and intravascular fluid redistribution resulting in the clinical manifestations of HELLP syndrome. As part of this it can be postulated that there may be damage to the endothelium of the vasa nervorum of the vestibulocochlear nerve, resulting in a hearing loss as demonstrated by the case described in this report. 4. Conclusion HELLP syndrome is a rare but life threatening complication of pregnancy. We have presented the case of a woman who developed sudden sensorineural hearing loss during the acute phase of her HELLP syndrome episode. More research is needed to fully understand the pathophysiology of the condition, but endothelial cell injury and the subsequent dysfunctional cascade of coagulation, vasoconstriction and intravascular fluid redistribution may lead to the clinical manifestation of hearing loss. In any patient with HELLP
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syndrome and sudden hearing loss, HELLP syndrome itself should be considered as the cause of the hearing loss. Funding This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. Conflict of interest There are no conflicts of interest to declare. References [1] Pritchard JA, Weisman Jr R, Ratnoff OD, Vosburgh GJ. Intravascular hemolysis, thrombocytopenia and other hematologic abnormalities associated with severe toxemia of pregnancy. N Engl J Med 1954;250:89e98. [2] Sibai BM, Barton JR. Expectant management of severe preeclampsia remote from term: patient selection, treatment, and delivery indications. Am J Obstet Gynecol 2007;196:514. e1-9. [3] Sheikh RA, Yasmeen S, Pauly MP, Riegler JL. Spontaneous intrahepatic hemorrhage and hepatic rupture in the HELLP syndrome: four cases and a review. J Clin Gastroenterol 1999;28:323e8. [4] Benedetto C, Marozio L, Tancredi A, Picardo E, Nardolillo P, Tavella AM, et al. Biochemistry of HELLP syndrome. Adv Clin Chem 2011;53:85e104. [5] Ruggenenti P, Remuzzi G. Pathophysiology and management of thrombotic microangiopathies. J Nephrol 1998;11:300e10. [6] Roberts JM, Taylor RN, Musci TJ, Rodgers GM, Hubel CA, McLaughlin MK. Preeclampsia: an endothelial cell disorder. Am J Obstet Gynecol 1989;161: 1200e4. [7] Altuntas¸ EE, Yenicesu AGI, Mutlu AE, Muderris S, Çetin M, Çetin A. An evaluation of the effects of hypertension during pregnancy on postpartum hearing as measured by transient-evoked otoacoustic emissions. Acta Otorhinolaryngol Ital 2012;32:31e6. [8] Rasmussen H. Sudden deafness. Acta Otolaryngol 1949;37:65e70. [9] Schreiber BE, Agrup C, Haskard DO, Luxon LM. Sudden sensorineural hearing loss. Lancet 2010;375:1203e11. [10] Stillman IE, Karumanchi SA. The glomerular injury of preeclampsia. J Am Soc Nephrol 2007;18:2281e4.